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Hematology > Hematology Exam 1 > Flashcards

Hematology Exam 1 Flashcards

1
Q

What percentage of our body is blood?

A

8% blood, 92% other fluids and tissues

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2
Q

What percentage of blood is plasma?

A

55% plasma, 45% formed elements

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3
Q

What percentage of plasma is water?

A

92% water, 6% proteins, 2% other solutes

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4
Q

How many platelets, leukocytes and erythrocytes are in formed elements of blood?

A

Platelets=140-340 thousand, Leukocytes=5-10 thousand, Erythrocytes=4.2-6.2 million

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5
Q

What makes up the 6% of proteins in plasma?

A

Albumins 58%, Globulins 38%, Fibrinogen 4%

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6
Q

What makes up the 2% of other solutes in plasma?

A

Ions, nutrients, waste products, gases, regulatory substances

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7
Q

What makes up the leukocytes in the formed elements of blood?

A

Neutrophils 54-67%, Lymphocytes 25-36%, Monocytes 3-8%, Eosinophils 1-4%, Basophils 0.75-1%. Never let my engine blow! 60, 30, 6, 3, 0!

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8
Q

Pluripotent stem cells –> committed myeloid stem cells –>

A

platelets and erythrocytes

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9
Q

Pluripotent stem cells –> committed myeloid stem cells –> myeloblasts –>

A

macrophages

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10
Q

Pluripotent stem cells –> committed myeloid stem cells –> myeloblasts –> promyeloblasts –>

A

neutrophils, eosinophils, basophils

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11
Q

Pluripotent stem cells –> committed lymphoid stem cells –>

A

Mature T-lymphocytes effector cell, natural killer cells, mature b-lymphocyte plasma cell

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12
Q

Platelet function steps

A

Exposure, adhesion, activation, aggregation, plug formation, clot retraction dissolution

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13
Q

Hemostasis, blood vessel injury neural pathway is?

A

BV injury – neural –> blood vessel constriction– reduced blood flow –> stable homeostatic plug

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14
Q

Hemostasis, blood vessel injury platelet function is?

A

BV injury – platelet activation –> primary hemostatic plug –> Plt Fusion

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15
Q

Hemostasis, blood vessel injury tissue factor pathway is?

A

BV injury – tissue factor –> coagulation activation – thrombin, fibrin –> stable hemostatic plug

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16
Q

How quickly is coagulation initiated in a normal individual?

A

Coagulation is initiated within 20 seconds after an injury occurs to blood vessel damaging the endothelial cells. Platelets immediately start to form a hemostatic plug at the site of injury.

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17
Q

Endothelin release in a blood vessel during BV injury causes…

A

vasoconstriction

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18
Q

Layers of blood vessel wall are?

A

Endothelium (inner), Basement membrane, Arteriole smooth muscle

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19
Q

What are the steps of primary hemostasis?

A

Platelet adhesion to vessel wall, platelet changes shape (flattens) on vessel wall, granule release from platelet into the blood (ADP, TXA2), recruitment of more platelets on to other platelets already on the wall, aggregation (hemostatic plug)

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20
Q

What is secondary hemostasis? What do the platelet granules do?

A

Plasma components called coagulation factors respond. They form fibrin strands- which strengthen the platelet plug. Platelets adhering to and activated by collagen in the blood vessel endothelium form plug. Activated platelets release the contents of their granules. These contain a variety of substances that stimulate further platelet activation and enhance the hemostatic process.

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21
Q

What are the steps of secondary hemostasis?

A

Tissue factor is released from the endothelium, phospholipid complex expression, thrombin activation, fibrin polymerization

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22
Q

What are intrinsic pathway problems?

A

Problems or substances in the blood itself, activates blood clotting cascade.

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23
Q

What are extrinsic pathway problems?

A

Means “outside” the blood, changes in blood vessels, tissue factor is released which begins the clotting cascade

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24
Q

What was the cascade pathway previously thought as?

A

consisted of 2 pathways of equal importance joined to a common pathway

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25
Q

What is the cascade pathway known now as?

A

Now known that the primary pathway for the initiation of blood coagulation is the tissue factor pathway.

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26
Q

The coagulation cascade of secondary homeostasis has 2 pathways that includes…–

A

The contact activation pathway (aka intrinsic pathway) and the tissue factor pathway (aka extrinsic pathway).

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27
Q

How does fibrinogen become fibrin?

A

Prothrombin —XaVa—> thrombin (a protease) meets with fibrinogen to become fibrin

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28
Q

What is the tissue factor pathway (extrinsic pathway)?

A

TF –> VIIa –> XaVa –> Thrombin a process by which thrombin, the most important constituent of the coagulation cascade in terms of its feedback activation roles, is released very rapidly. FVIIa circulates in a higher amount than any other activated coagulation factor.
Following damage to the blood vessel, FVII leaves the circulation and comes into contact with tissue factor (TF) expressed on tissue-factor-bearing cells (stromal fibroblasts and leukocytes), forming an activated complex (TF-FVIIa).

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29
Q

What is the contact activation pathway (intrinsic pathway)?

A

XIIa –> XIa –> IXa -VIIa –> XaVa –> Thrombin <– Prothrombin. The contact activation pathway begins with formation of the primary complex on collagen by high-molecular-weight kininogen (HMWK), prekallikrein, and FXII (Hageman factor). Prekallikrein is converted to kallikrein and FXII becomes FXIIa. FXIIa converts FXI into FXIa. Factor XIa activates FIX, which with its co-factor FVIIIa form the tenase complex, which activates FX to FXa. The minor role that the contact activation pathway has in initiating clot formation can be illustrated by the fact that patients with severe deficiencies of FXII, HMWK, and prekallikrein do not have a bleeding disorder. Instead, contact activation system seems to be more involved in inflammation.

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30
Q

What is the final common pathway?

A

Fibrinogen –> Fibrin (soft clot) – XIIIa –> Fibrin (hard clot)

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31
Q

How does plasminogen become plasmin?

A

Plasmin is released as a zymogen called plasminogen (PLG) from the liver into the systemic circulation. Plasminogen is the inactive precursor of the trypsin-like serine protease plasmin. It is normally found circulating through the blood stream. When plasminogen becomes activated and is converted to plasmin, it unfolds a potent enzymatic domain that dissolves the fibrinogen fibers that entgangle the blood cells in a blood clot. This is called fibrinolysis.

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32
Q

What enzymes help with the conversion of plasminogen to plasmin?

A

In circulation, plasminogen adopts a closed, activation resistant conformation. Upon binding to clots, or to the cell surface, plasminogen adopts an open form that can be converted into active plasmin by a variety of enzymes, including tissue plasminogen activator (tPA), urokinase plasminogen activator (uPA), kallikrein, and factor XII (Hageman factor). Fibrin is a cofactor for plasminogen activation by tissue plasminogen activator. Urokinase plasminogen activator receptor (uPAR) is a cofactor for plasminogen activation by urokinase plasminogen activator. The conversion of plasminogen to plasmin involves the cleavage of the peptide bond between Arg-561 and Val-562.

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33
Q

What does plasmin become?

A

Fibrin—> fibrin degradation products

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34
Q

What are the extrinsic and intrinsic pathways that convert plasminogen to plasmin?

A

Extrinsic=damaged endothelial cells –> t-PAI –> plasminogen. Intrinsic=damaged endothelial cells –> Factor XIIa –> urokinase –> plasmin –> fibrin –> fibrin degradation products

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35
Q

What do you ask concerning blood when taking history?

A

Personal history: miscarriages, menorrhagia, nose bleeds, bleeding with dental work. Family history and genetic risk: miscarriages. Known liver, immunologic or hematologic conditions. Chronic health problems. Medications (prescription, OTC and herbals). Occupations, hobbies (exposure to hematologically damaging agents). Dietary history. Socioeconomic status.

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36
Q

What body systems do hematologic problems affect?

A

skin, mucous membranes, lymph nodes, cardiopulmonary, renal, MSK, abdominal, CNS

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37
Q

Lab diagnostics- What do you check for in a coagulation evaluation?

A

CBC, bleeding time, prothrombin/INR, partial thrombin time, active PTT (aPTT)

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38
Q

What do you check for in a anemia evaluation?

A

Iron, ferritin, transferrin, total iron binding capacity, direct and indirect coomb’s test, reticulocyte count

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39
Q

Prothrombin time/INR measures what?

A

Measures the coagulation effectiveness of prothrombin, fibrinogen, factors V, VII, X and vitamin K (extrinsic and common pathway). Deficiency of clotting factors=PT is increased!

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40
Q

International normalized ratio (INR) range is what?

A

Same regardless of testing reagents or methods
INR therapeutic ranges from 1.5 to 3.5 depending on the problem being treated. International Sensitivity Index (ISI), Usually between 1.0-2.0.

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41
Q

What are the specific preferred INR values?

A

DVT prophylaxis=1.5-2.0. DVT treatment=2.0-3.0. Orthopedic Surgery=2.0-3.0. Embolus Prevention Atrial Fibrillation=2.0-3.0. Pulmonary Embolism Treatment=2.5-3.5. Prosthetic Valve Embolus Prevention=2.5-3.5.

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42
Q

What does partial thromboplastin time measure (PTT)?

A

Coagulation effectiveness of clotting factors of the intrinsic pathway. Factors II,V, VIII, IX, XI and XII. Factors II, IX and X depend on vitamin K- liver disease can decrease their levels-prolonging PTT.
Normal 60-70 seconds. Critical >100 seconds.

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43
Q

Which drugs disrupt platelet action?

A

Impairs platelet ability to clump together to control bleeding= Vit K, ASA, NSAIDS, Heparin, Coumadin, Warfarin, tPA.

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44
Q

What are some vascular disorders?

A

scurvy, easy bruising, henoch-schonlein purpura

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45
Q

What are some platelet disorders?

A

Quantitative-thrombocytopenia, Qualitative-platelet function disorders

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46
Q

What are some coagulation disorders?

A

Congenital- haemophilia (A, B), von willebrands. Acquired- vitamin K deficiency, liver disease. Mixed/consumption: DIC

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47
Q

Factor VIII: Hemophilia A Deficiency- how common is it? How is it acquired?

A

Most common type of hemophilia. AKA ‘classic hemophilia.’ Primarily an inherited disorder in which one of the proteins needed to form blood clots is missing or reduced. Approximately one in 5,000 males in the US.

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48
Q

Does someone with factor VIII hemophilia A bleed faster or longer than a normal person?

A

Patient does not bleed harder or faster than a person without hemophilia, they bleed longer.
Normal plasma levels of FVIII range from 50% to 150%. Different levels of hemophilia: mild, moderate, and severe.

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49
Q

How much clotting factor is in mild factor VIII hemophilia A?

A

6% up to 49% of the normal clotting factor in their blood

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50
Q

How much clotting factor is in moderate factor VIII hemophilia A?

A

15% of the hemophilia population- 1% up to 5% of the normal clotting factor in their blood

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51
Q

How much clotting factor is in severe factor VIII hemophilia A?

A

60% of the hemophilia population- <1% of the normal clotting factor in their blood

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52
Q

What is genetic counseling for factor VIII hemophilia A based on?

A

family pedigree

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53
Q

Which chromosome is the factor VIII hemophilia A gene located on?

A

X chromosome

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54
Q

What are the 4 possible outcomes of a woman who is a carrier for factor VIII hemophilia A, repeated for each and every pregnancy?

A
  1. A girl who is not a carrier, 2. A girl who is a carrier, 3. A boy without hemophilia, 4. A boy with hemophilia
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55
Q

What are the treatments for mild factor VIII hemophilia A?

A

Desmopressin acetate (DDAVP) to treat small bleeds. Deep cuts or internal bleeding ,bleeding into the joints or muscles, require more complex treatment. The clotting factor missing (VIII or IX) must be replaced.

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56
Q

What are the treatments for moderate to severe factor VIII hemophilia A?

A

Factor VIII concentrate. Prophylaxis w/ plasma or recombinant factor IV ( can be self injected).

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57
Q

How common is Hemophilia B (factor IX) Christmas disease? What is missing in this disease?

A

Second most common, protein needed to form clots reduced/missing, 70% have family history, 30% spontaneous mutation, 1 in 25,000 male births, bleeds longer.

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58
Q

How common is mild Hemophilia B?

A

5-50%

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59
Q

How common is moderate Hemophilia B?

A

1-5%

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60
Q

How common is severe Hemophilia B?

A

< 1%

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61
Q

How common is normal F IX?

A

50-150%?

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62
Q

How do you treat Hemophilia B, Factor IX?

A

plasma and recombinant

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63
Q

What is Factor XI, Hemophilia C also known as? How common is it?

A

Also known as: Plasma Thromboplastin Antecedent (PTA) Deficiency, Rosenthal Syndrome.
1 in 100,000 people, Autosomal dominant, males=females, More common in Ashkenazi Jewish descent.

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64
Q

What tests do you use to diagnose Factor XI, Hemophilia C?

A

Bleeding time test, platelet function tests, prothrombin time (PT), activated partial thromboplastin time (aPTT) tests.Specific Factor XI assay is extremely useful in ruling out combined deficiencies. There is variability in the presentation of this disease. XI part of clotting cascade.

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65
Q

How does Hemophilia C, Factor XI present?

A

Not likely to bleed spontaneously, Hemorrhage normally occurs after trauma or surgery.Joint bleeds are uncommon. Prone to bruising, nosebleeds, or blood in the urine.Woman may experience menorrhagia and prolonged bleeding after childbirth.

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66
Q

What is the treatment for Hemophilia C, Factor XI?

A

In the US no factor XI concentrates available until this year. Fresh-frozen plasma is normally used for treatment. But since Factor XI is not concentrated in fresh frozen plasma, considerable amounts of plasma may be required to maintain the factor level. In the case of mouth bleeds antifibrinolytic products such as Amicar are used.

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67
Q

What are other blood clotting factors are there?

A

I, II, V, X, XII, XIII

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68
Q

Factor I is?

A

fibrinogen

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69
Q

Factor II is?

A

prothrombin

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70
Q

Factor V is?

A

proaccelerin, labile factor

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71
Q

Factor VI is?

A

thromboplastin antecedent

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72
Q

Factor XII is?

A

Hageman factor

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73
Q

Factor XIII is?

A

fibrin-stabilizing factor

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74
Q

Factor VII is?

A

stable factor

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75
Q

Factor VIII is?

A

antihemophilic factor

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76
Q

Factor IX is?

A

christmas factor

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77
Q

Factor X is?

A

Stuart-prower factor

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78
Q

Factor XI is?

A

plasma factor

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79
Q

What are some disorders of Factor I, fibrinogen deficiency?

A

afibrinogenemia, dysfibrinogenemia, or hypofibrinogenemia, 1 per 2 million people, autosomal recessive, females and males equally

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80
Q

How is a diagnosis made for disorders of Factor I, fibrinogen deficiencies?

A

Often at birth, spontaneous abortion, more disposed to thrombosis. Labs: measure the amount of fibrinogen in the blood, prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin clotting time (TCT) test.

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81
Q

How do you treat disorders of Factor I, fibrinogen deficiencies?

A

cryoprecipitate

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82
Q

How common is Factor II, prothrombin deficiency?

A

2%-50%, >50% little or no bleeding disorders, Rare (26 cases), Autosomal recessive males=females

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83
Q

What labs do you use to diagnose Factor II, prothrombin deficiency?

A

prothrombin time (PT), activated partial thromboplastin time (aPTT)

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84
Q

How do you treat Factor II, prothrombin deficiency?

A

Fresh Frozen Plasma, Prothrombin complex concentrates (PCCs) (May precipitate thromboembolic events)

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85
Q

What are some disorders of Factor V, proaccelerin/labile factor deficiency?

A

Owren’s disease, labile factor deficiency, proaccelerin deficiency’ parahemophilia. (-Not to be confused with Factor V Leiden- a type of thrombophilia). 1 per million get it. Autosomal recessive : males=females. Catalyst or “accelerator” in the process to convert prothrombin to thrombin.

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86
Q

How does mild Factor V, proaccelerin/labile factor deficiency present?

A

easy bruising, nose or mouth bleeds, bleeding after trauma or surgery

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87
Q

How does severe Factor V, proaccelerin/labile factor deficiency present?

A

joint bleeding, gastrointestinal bleeds, hematomas

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88
Q

How does Factor V, proaccelerin/labile factor deficiency present in women?

A

heavy menstrual bleeding (menorrhagia), first trimester miscarriage other complications during pregnancy and delivery

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89
Q

How do you diagnose Factor V, proaccelerin/labile factor deficiency?

A

Diagnosis is made through personal & family history. Labs: prothrombin time (PT) test, partial thromboplastin time (PTT), activated partial thromboplastin time (APTT) test, confirmed by a factor X functional activity (FX:C) or Russell viper venom (RVV) time assay.

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90
Q

How common is Factor X, Stuart-Prower factor deficiency? What does factor X do?

A

Incidence:1 in 500,000-1,000,000 world wide, Autosomal recessive, Affects males/females equally, Factor X activates enzymes that form clot

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91
Q

How do you treat Factor X, Stuart-Prower factor deficiency? What about a mild case?

A

There is no factor X concentrate currently available in the US. Fresh-frozen plasma or plasma-derived Prothrombin Complex concentrates (PCCs) are normally used as treatment.Fibrin glue may relieve bleeding symptoms. Mild: antifibrinolytic agents, Aminocaproic acid or tranexamic acid topical therapies.

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92
Q

Do you need treatment for a Factor XII: Hageman factor deficiency?

A

Normally do not require treatment.Having a low factor XII level has little to no clinical significance.

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93
Q

How common and what are the symptoms for a Factor XII, Hageman factor deficiency?

A

Incidence of Factor XII deficiency: 1 in 1 million.
Autosomal recessive fashion, males=females
Usually do not experience bleeds.

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94
Q

What is the rarest of all factor deficiencies?

A

Fibrin Stabilizing Factor deficiency, Factor XIII.
Autosomal recessive : males=females. Protein responsible for stabilizing the formation of a blood clot. Usually associated w/ trauma. High risk of head bleeds w/ or w/out trauma. Delayed bleeding after surgery,

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95
Q

How do you diagnose Factor XIII, hageman deficiency?

A

normal coagulation screening tests, detailed family history., specific factor XIII assays can confirm the diagnosis

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96
Q

What tips should you give to someone who is at risk for bleeding excessively?

A

Goal is to limit trauma that can lead to bleeding.
Use soft-bristled toothbrush, electric shaver, avoid aspirin or aspirin-containing products. No contact sports or activities that may result in impact, scratches or abrasions. If bumped, apply ice to area for 1 hour. Avoid straining for BM – use stool softener, no enemas, rectal suppositories or anal sex. Avoid blowing nose.

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97
Q

What is the platelet count for someone with thrombocytopenia?

A

Defined as a platelet count of less than 150 × 103 per μL.

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98
Q

What is the etiology of thrombocytopenia and how is it usually discovered?

A

Often discovered incidentally when obtaining a CBC. The etiology usually is not obvious. Requires additional investigation.

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99
Q

What platelet count rarely has symptoms in thrombocytopenia?

A

Platelet counts > 50 × 103 per μL rarely have symptoms.

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100
Q

What platelet count may manifest as purpura in thrombocytopenia?

A

Platelet count from 30 to 50 × 103 per μL may manifest as purpura.

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101
Q

What platelet count may cause bleeding with minimal trauma in thrombocytopenia?

A

10 to 30 × 103 per μL may cause bleeding with minimal trauma.

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102
Q

What platelet count may cause spontaneous bleeding and constitutes a hematologic emergency in thrombocytopenia?

A

< 5 × 103 per μL may cause spontaneous bleeding and constitutes a hematologic emergency.

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103
Q

Does thrombocytopenia ever require inpatient treatment?

A

emergent thrombocytopenia does, nonemergent just needs outpatient treatment

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104
Q

Isolated thrombocytopenia is associated with?

A

drug-induced thrombocytopenia, immune thrombocytopenic purpura (ITP) (Idiopathic thrombocytopenia purpura), pseudothrombocytopenia

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105
Q

Multi system thrombocytopenia is shown in what conditions?

A

acute infection, heparin-induced thrombocytopenia,
liver disease, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, disseminated intravascular coagulation (DIC) or a hematologic disorder.

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106
Q

How will thrombocytopenia present in pregnancy?

A

gestational thrombocytopenia, preeclampsia, HELLP (hemolysis, elevated liver enzymes, and low platelet count)

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107
Q

What should you ask about in a history for someone with suspected thrombocytopenia?

A

Easy bruising, petechiae, melena, rashes fevers and bleeding. Medications, immunizations, recent travel, recent hospitalization, transfusion history, family history, medical history, and acute/chronic alcohol use.

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108
Q

What should you ask about in a history for a pregnant woman with suspected thrombocytopenia?

A

visual symptoms, headaches, abdominal pain, influenza-like symptoms

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109
Q

What should you look for during physical exam for someone with suspected thrombocytopenia?

A

Skin-petechiae, purpura, bruising. Eyes- hemorrhage is suggestive of central nervous system bleeding. Lymph nodes, Abdomen-splenomegaly, hepatomegaly. Neurologic system.

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110
Q

What labs should you get for someone with suspected thrombocytopenia?

A

CBC, a peripheral blood (white blood cells disorders, hemolytic anemias, thrombocytopenia), Coagulation studies, LFTs, kidney function

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111
Q

What are the etiologies for decreased platelet production?

A

Bone marrow failure (aplastic anemia, paroxysmal nocturnal hemoglobinuria, shwachman-diamond syndrome). Bone marrow suppression (medication, chemotherapy, irradiation), chronic alcohol abuse, congenital macrothrombocytopenias (alport syndrome, bernard -soulier syndrome, fancoli anemia, platelet-type or pseudo-von willebrand disease, wiskott-aldrich syndrome), Myelodysplastic syndrome, neoplastic marrow infiltration, nutritional deficiencies (vitamin B12, folate).

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112
Q

What are the etiologies for increased platelet consumption?

A

Alloimmune destruction (posttransfusion, neonatal, posttransplantation), Autoimmune syndromes (antiphospholipid syndrome, SLE, sarcoidosis, Disseminated intravascular coagulation/severe sepsis, Drug-induced thrombocytopenia.

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113
Q

What are sequestration or other etiologies for thrombocytopenia?

A

chronic alcohol abuse, dilutional thrombocytopenia (hemorrhage, excessive crystalloid infusion), gestational thrombocytopenia, mechanical destruction (aortic valve, mechanical valve, extracorporeal bypass), preeclampsia/HELLP syndrome, Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, Hypersplenism, distributional thrombocytopenia, Liver disease, cirrhosis, fibrosis, portal hypertension, Pseudothrombocytopenia, Pulmonary emboli and Pulmonary hypertension.

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114
Q

Idiopathic thrombocytopenia (ITP) is also called?

A

autoimmune thrombocytopenia- body forms antibodies to platelets and then begins to destroy them

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115
Q

What are the signs and symptoms of ITP?

A

Abrupt onset (childhood ITP), Gradual onset (adult ITP), Purpura, Menorrhagia, Epistaxis, Gingival bleeding, Recent live virus immunization -childhood ITP, Recent viral illness and Bruising tendency.

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116
Q

What viral infections are associated with thrombocytopenia?

A

Cytomegalovirus, Epstein-Barr virus, hepatitis C virus, HIV, Mumps, parvovirus B19, Rickettsia
rubella, and varicella-zoster virus.

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117
Q

Besides seeing decreased platelets in your labs, what else could you do to confirm thrombocytopenia?

A

It depends on the suspicion, but smear and coagulation studies and liver/kidney function tests could help.

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118
Q

How do you treat thrombocytopenia?

A

Referral to hematologist, interventions are corticosteroids, IV immunoglobulin, and chemotherapy.

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119
Q

How will thrombotic thrombocytopenic purpura (TTP) present?

A

Acute or subacute onset of symptoms: Neurologic dysfunction, Anemia, Thrombocytopenia, Severe bleeding is unusual, petechiae are common, Fever - 50% of Pts, Dark urine (hemoglobinuria).

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120
Q

In full blown TTP, what pentad will you see?

A

hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever and renal disease.

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121
Q

How is hemolytic uremic syndrome different from TTP?

A

Less CNS involvement than TTP, more severe renal involvement in HUS. In HUS they will have a hx of diarrhea.

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122
Q

What important labs do you get for a suspected thrombocytopenia condition?

A

CBC, Platelet count, Blood smears, Coagulation studies, BUN creatinine, Serum bilirubin and Lactate dehydrogenase.

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123
Q

What are schistocytes?

A

red blood cell fragments

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124
Q

How do you treat TTP?

A

Admit them, stabilize them, refer to hematologist, plasma exchange with frozen fresh plasma, octaplas (octapharma), corticosteroids

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125
Q

What is von willebrand factor?

A

a protein that acts like glue to help the platelets stick together and form a blood clot

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126
Q

Which clotting factor does von willebrand factor carry?

A

clotting factor VIII

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127
Q

Von willebrand factor disease is more common and milder than hemophilia, true or false?

A

TRUE

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128
Q

What are the characteristics of type 1 VWD and how common is it?

A

Low levels of von willebrand factor and may have low levels of factor VIII. Type 1 is the mildest and most common form of VWD. About 3/4 people who have VWD have type 1.

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129
Q

What subtypes are there for type 2 VWD?

A

2A, 2B, 2M and 2N. This factor doesn’t work well. Type 2 is based on different gene mutations.

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130
Q

What are the characteristics of type 3 VWD and how common is it?

A

NO von willebrand factor levels and low levels of factor VIII. It is the most serious form and it is very rare.

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131
Q

What are the signs and symptoms of type 1 and 2 VWD?

A

Frequent, large bruises from minor bumps or injuries, Frequent or hard-to-stop nosebleeds
Prolonged bleeding from the gums after a dental procedure, Heavy or prolonged menstrual bleeding in women, Blood in stools, Blood in urine, Heavy bleeding after a cut or other accident, Heavy bleeding after surgery and Heavy bleeding after delivery.

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132
Q

What are the signs and symptoms of type 3 VWD?

A

All of the same signs and symptoms for type 1 and 2, plus severe bleeding episodes for no reason. Can be fatal if not treated right away.

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133
Q

What should you ask for in the history for someone with suspected VWD?

A

Bleeding from a small wound that lasted more than 15 minutes or started up again within the first 7 days following the injury. Prolonged, heavy, or repeated bleeding that required medical care after surgery or dental extractions. Bruising with little or no apparent trauma, there may be a lump under a bruise. Nosebleeds for no reason and lasted more than 10 minutes despite pressure on the nose. Any blood in stools for no reason. Heavy menstrual bleeding (bleeding with clots or lasts longer than 7-10 days). History of muscle or joint bleeding.

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134
Q

What medications should you ask if they’re taking and what conditions in their past medical history should you ask about for someone with suspected VWD?

A

Aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), clopidogrel (Plavix), Warfarin, heparin. Liver or kidney disease, blood or bone marrow disease, high or low blood platelet counts.

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135
Q

What should you look for during the physical exam for someone with suspected VWD?

A

Skin - petechiae, purpura, bruising, Eyes- hemorrhage is suggestive of central nervous system bleeding, lymph nodes, Abdomen-splenomegaly, hepatomegaly, neurologic system.

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136
Q

What tests do you use to diagnose VWD?

A

based on suspicion, presentation and history. No single test can diagnose VWD. There is a VWB factor antigen. VWB factor ristocetin (ris-to-SEE-tin) cofactor activity. Factor VIII clotting activity. VWB factor multimers.
Platelet function test. Test more than once to confirm a diagnosis. Refer to a hematologist to confirm the diagnosis and followup.

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137
Q

How do you treat VWD?

A

Based on the type of VWD and how severe it is.
Most cases of VWD are mild, and may need treatment only for surgery, tooth extraction, or an accident. Medications-Desmopressin,
Antifibrinolytic medicines, Fibrin glue placed directly on a wound to stop bleeding. Von Willebrand factor replacement therapy.
IV infusion of concentrated von Willebrand factor and factor VIII.

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138
Q

What are the specific treatments for women with VWD?

A

Oral contraceptives, A levonorgestrel intrauterine device. Contains the hormone progestin. Aminocaproic acid or tranexamic acid.
antifibrinolytic medicines reduce bleeding by slowing the breakdown of blood clots. Desmopressin. Women who are done having children or don’t want children=endometrial ablation.Hysterectomy=Not an indication but if done for other reasons, but there is a possible bleeding complication during surgery.

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139
Q

What factor and mutation is associated with thrombophilia?

A

Factor V Leiden thrombophilia and Prothrombin G20210A mutation

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140
Q

What are the rare conditions associated with thrombophilia?

A

Protein C and protein S. Antithrombin III deficiencies.

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141
Q

What is the most common inherited form of thrombophilia?

A

Factor V Leiden Thrombophilia. Inherited disorder of blood clotting. Factor V Leiden is the name of a specific gene mutation that results in increased tendency to form abnormal blood clots (DVTs are common). Most common inherited form of thrombophilia esp in European descent.

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142
Q

Where will clots form in Factor V Leiden Thrombophilia?

A

DVTs occur most often in the legs or pelvis
(also occur in the brain, eyes, liver, and kidneys).
Increased risk of clots will break away, (travel through the bloodstream, PE could form).

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143
Q

What are some complications for women with Factor V Leiden thrombophilia?

A

Increased risk of spontaneous abortion
(Three x more likely to have recurrent miscarriages in 2 or 3 trimester), More likely to develop clots on combination oral contraceptives, May be associated w/ preeclampsia, slow fetal growth, and abruption.

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144
Q

How does G20210A mutation present?

A

Same presentation and risks as Factor V Leiden thrombophilia. They are both detected by PCR. Screening is not cost effective for either.

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145
Q

What is DIC (disseminated intravascular coagulation)?

A

Excessive clotting that uses up all platelets and clotting factors resulting in internal and/or external bleeding

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146
Q

What are the etiologies for DIC?

A

Sepsis, Hemorrhage, Cancer, Complications of pregnancy or childbirth, Trauma, Surgery, Poisonous snakes, Burns and Frostbite.

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147
Q

What are the differences between acute and chronic DIC?

A

Acute-develops quickly and must be treated ASAP. Chronic-develops slowly, it is the most common cause (cancer) and there is more clotting than bleeding.

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148
Q

What is the function of the spleen?

A

stores WBC for fighting infection, filters bacteria, destroys old or imperfect RBCs

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149
Q

How does the liver contribute to blood?

A

produces clotting factors, stores blood cells, converts bilirubin to bile, stores extra iron

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150
Q

How long is the life span for an erythrocyte?

A

120 days

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151
Q

What stain is used for reticulocytes?

A

Uses supravital stain which stains cells in the living state.

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152
Q

What are the formulas for reticulocyte % and corrected retic?

A

retics per 1,000 RBC’s/10= Retic %, % retics x pt. HCT/45= Corrected retic

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153
Q

What are some sources of error in the lab regarding blood?

A

Inadequate mixing of specimen, Hemolyzed specimens, Lipemic specimens, Cold agglutinins, Clotted specimens, Platelet clumps, Diluted specimens

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154
Q

Heme portion of hemoglobin has an iron portion and transports up to how many molecules of oxygen?

A

4

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155
Q

What does the globin portion do in hemoglobin?

A

carries carbon dioxide and helps with acid-base balance

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156
Q

Where is iron absorbed in the body?

A

Supplied by the diet and absorbed in the duodenum and proximal jejunum

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157
Q

What carries iron and what does iron bind to?

A

Carried by protein transferrin to the cells and binds to the heme portion of the hemoglobin and it is recycled

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158
Q

What is the most numerous blood cell?

A

erythrocytes 4-6 million

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159
Q

What does a red blood cell not have in mammals but us present in red blood cells in other vertebrates?

A

In man and in all mammals, erythrocytes are devoid of a nucleus and have the shape of a biconcave lens. In the other vertebrates (e.g. fishes, amphibians, reptilians and birds), they have a nucleus.

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160
Q

What is mostly responsible for carrying CO2 in blood?

A

carried by plasma in the form of soluble carbonates

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161
Q

Why is it beneficial to not have a nucleus in a red blood cell?

A

Lack of nucleus allows more room for hemoglobin and the biconcave shape of these cells raises the surface and cytoplasmic volume ratio. Characteristics make more efficient the diffusion of oxygen by these cells.

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162
Q

At the end of 120 days, what happens to red blood cells?

A

mean life of erythrocytes is about 120 days. When they come to the end of their life, they are retained by the spleen where they are phagocyted by macrophages

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163
Q

What are the different shapes of red blood cells?

A

red cells can have different shapes: normal (discocyte), berry (crenated), burr (echinocyte), target (codocyte), oat, sickled, helmet, pinched, pointed, indented, poikilocyte

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164
Q

What is hematocrit?

A

whole blood occupied by intact red blood cells

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165
Q

What causes anemia (red blood cell deficiency)?

A

Blood loss -which is the most common cause, can be short term or chronic. Iron deficiency anemia,
heavy menstrual periods, surgery, trauma, GI/GU bleed and cancer, obvious bleeding, occult bleeding, repeated diagnostic tests. Lack of red blood cell production (acquired from poor diet -lack of Vit C, Fe, folic acid, Vit B12, Zn (or malabsorption) abnormal hormone levels- erythropoietin).

166
Q

What is dependent on red blood cell mass?

A

Hb, Hct, and RBC are all concentrations dependent on red blood cell mass as well as plasma volume. All 3 values will be reduced if the red blood cell mass (RBCM) is reduced or the plasma volume is increased.

In acute bleeding there is acute volume depletion. It is not until the fluid is moved into the intravascular space that lab results will reflect blood loss- 36-48hrs later. Volume depletion may mask low Hb/Hct until volume depletion is corrected.

167
Q

Who is at risk for anemia?

A

Women of childbearing age because they have no iron stores therefore any bleeding puts them at risk, children, older adults and the malnourished.

168
Q

What causes dilutional anemia?

A

Pregnancy -the plasma volume increases by 25-50%

169
Q

Anemias can be classified according to the mean corpuscular volume (MCV) into…

A

Microcytic, normocytic, and macrocytic. Microcytic anemia is defined by a MCV of < 80. The differential diagnosis of a microcytic anemia includes iron deficiency anemia (IDA), thalassemias, anemia of chronic disease (ACD) and sideroblastic anemias, including lead poisoning.

170
Q

What are the signs and symptoms of anemia?

A

Depend on the severity and rate of development-
Symptoms are less likely if it evolves slowly due to compensatory mechanisms. Can result from decreased oxygen delivery and/or hypovolemia.
Headache, dizziness, shortness of breath, pale skin, coldness in the feet and hands, chest pain, bounding pulse, roaring in the ears, because the heart has to work harder to pump oxygen rich blood.

171
Q

Erythropoiesis occurs in the bone marrow when it is stimulated by _________, erythroid specific growth factor and erythropoietin

A

cytokines

172
Q

What is erythropoietin?

A

Erythropoietin is an endocrine hormone made in the kidneys and enhances the maturity of normoblasts which turn into reticulocytes. After roughly 4 days the reticulocyte turns into a mature RBC. This RBC circulates for 110 to 120 days until macrophages remove them.

173
Q

Reticulocytes remain in the blood for ____ day before maturing to RBC’s

A

one

174
Q

What might you see on physical exam for someone with anemia?

A

Look for signs of organ or multisystem involvement
Tachycardia, dyspnea, fever or postural hypotension. Jaundice may be difficult to detect under artificial lighting. Pallor in the palms, nail beds, face or conjunctivae as a predictor varies from 19-70%. Watch for weight loss, wasting, poor muscle tone/strength to show severity/chronicity of disease. Lymphadenopathy, hepatosplenomegaly, bone tenderness especially over the sternum and bone pain. Petechiae, ecchymoses and other signs of coagulopathy. When ordering labs (CBC) order them STAT which should get results in 4 to 6 hours. Have the patient call you back in 24 hours for plan.

175
Q

What should you do when you refer someone with anemia?

A

Call the consultant for referral in advance of the patient appointment (i.e. Mrs. Jones with low WBC, axillary node, weight loss is coming in 3 days) and send results.

176
Q

What can you do to aid in the diagnosis for anemia?

A

You can do a chest x-ray, US/CT of the abdomen/pelvis to check for mediastinal/hilar nodes, enlarged liver or spleen or abdominal/pelvic nodes to aid in diagnosis.

177
Q

What labs should you order for someone with suspected anemia besides a CBC?

A

CBC which includes Hb, Hct, RBC, RBC indices and WBC. A WBC differential, PLT and reticulocyte count are not part of the routine CBC and would need to be ordered. Many machines will report RBC distribution width (RDW) but this is not enough to form any diagnosis. Blood smears should be evaluated by experienced examiners, not machines for differential diagnosis.

178
Q

What three RBC indices are measured in someone with suspected anemia?

A

Mean corpuscular volume (MCV)
(High values are almost exclusively seen in vitamin B12 or folate deficiency, High values also seen with medications and increased reticulocyte count, False positive results can be due to cold agglutins. Repeat tests should be brought to body temp before running).
Mean corpuscular hemoglobin (MCH)
(Low values are seen in thalassemia and iron deficiency, High values in macrocytosis of any cause), and Mean corpuscular hemoglobin concentration, (high values are almost exclusive to congenital or acquired spherocytosis or other congenital diseases where RBC’s are abnormally desiccated- SCD, Hb C disease, xerocytosis)

179
Q

What does a high level of reticulocytes mean in anemia?

A

high level with anemia reflects increased erythropoietic response to hemolysis or blood loss

180
Q

What does a low level of reticulocytes mean in anemia?

A

low level with anemia is strong evidence for deficient production (reduced marrow response)

181
Q

What does a low reticulocyte count with pancytopenia indicate? What about an extremely low count?

A

Low count with pancytopenia suggests aplastic anemia. Extremely low count suggests pre red cell aplasia.

182
Q

What does a low WBC count with anemia indicate?

A

Low WBC and anemia is indicative of bone marrow suppression or deficiencies of cobalamin or folate

183
Q

What does high WBC count with anemia indicate?

A

High WBC and anemia may indicate infection, inflammation or malignancy

184
Q

Nucleated RBCs are not normally found. In what abnormalities may you see them?

A

Their presence may mean malignancy, hematologic disease or severe sepsis or heart failure.

185
Q

What does a low platelet count indicate?

A

Low counts can indicate hypersplenism, autoimmune plt destruction, sepsis or folic acid/cobalamin deficiency

186
Q

What does a high platelet count indicate?

A

High counts may reflect chronic iron deficiency, myeloproliferative or myelodysplastic disorders

187
Q

When would you see pancytopenia?

A

Myelodysplasias, fibrosis, neoplasm, aplastic anemia, autoimmune – SLE, rheumatoid arthritis
Infectious – EBV, CMV, Chemotherapy and other medications, Vitamin deficiency- Vit B12 and folate

188
Q

Any abnormal blood lab requires what?

A

follow up with a blood smear

189
Q

When evaluating abnormal blood levels, why would measuring the rate of fall of Hb/Hct be helpful?

A

If Hb was not being replaced the resultant decrease would be 1% per day because there is only 1% reticulocytes in the blood. Therefore if the rate of fall is 15g/dl in 1 week that is a greater drop and therefore the patient must be bleeding or RBC are being destroyed.

190
Q

What will you see in labs for hemolysis?

A

Has a rapid fall in Hb, reticlocytosis, abnormally shaped RBC on smear, increased LDH and reduced haptoglobin are 90%specific for diagnosing hemolysis.

191
Q

When would you do a bone marrow exam?

A

It is not useful in anemia diagnosis. It is used in aplastic anemia, abnormal cells such as blasts, and pancytopenia.

192
Q

Chronic hemolytic anemia may worsen after…?

A

infection

193
Q

What could make autoimmune hemolytic anemia worse?

A

glucocorticoids

194
Q

What 2 other pathologies do you see commonly with anemia? (all three at the same time)

A

CHF and renal failure

195
Q

Because the RBC carries oxygen, the cardinal symptom of anemia is ….

A

fatigue

196
Q

What is aplastic anemia?

A

Diminished or absent hematopoietic cells in the marrow, (injury to pluripotent stem cells, it involves more than RBC’s, it is associated with pancytopenia
bone marrow failure is a more appropriate term)

197
Q

Is aplastic anemia microcytic, macrocytic or normocytic?

A

Normocytic, because the cells are normal size and shape, just inadequate in numbers. Pancytopenia in the absence of splenomegaly.

198
Q

What do you call congenital aplastic anemia?

A

fanconi’s anemia

199
Q

How do you acquire aplastic anemia?

A

Exposure to drugs, viruses, radiation or toxins, Malignancy, Autoimmune, or Pregnancy

200
Q

Which drugs could cause aplastic anemia?

A

NSAIDS- phenylbutazone which is now used for horses. Chloramphenicol – used for resistant infection. Gold – arthritis. Antiepileptics – felbamate, carbamazepine, valproic acid, phenytoin
and Nifedipine.

201
Q

What exposures can cause aplastic anemia?

A

Exposure to solvents, degreasing agents, pesticides and radiation are all factors
(Landscapers, shoe repair, benzene exposure, rubber factories, oil refineries, bus depot
Reduced ability to detoxify our environment may be a contributing factor, Use of non-bottled water increases risk)

202
Q

What viral exposures can cause aplastic anemia?

A

Epstein- Barr, Seronegative hepatitis, HIV, Herpes virus

203
Q

How does someone get autoimmune aplastic anemia?

A

Hypothesis is damage from drugs, radiation etc. stimulates lymphocyte activity which results in hematopoietic inhibitory response. This triggers cytokine cascade leading to apoptopic death of pluripotent cells. Stressed, dying and immunologically activated target cell population is seen.This is T cell mediated.

204
Q

What is the first line therapy for autoimmune aplastic anemia?

A

immunosuppressive therapy

205
Q

What are some forms of congenital aplastic anemia?

A

Paroxysmal nocturnal hemoglobinuria (These patients are prone especially after immunotherapy),
Fanconi’s anemia. (Diagnosed before 16yrs,
Has increased chromosomal breakage in lymphocytes on bone marrow examination)
Telomerase mutations, abnormal thrombopoietin

206
Q

What are the clinical manifestations for aplastic anemia?

A

Related to pancytopenia, not just anemia, fatigue and cardiopulmonary symptoms, recurrent infections due to leukopenia, mucosal hemorrhage and increased menstrual flow from thrombocytopenia, pallor and petichiae, liver, spleen and lymph nodes are not enlarged

207
Q

What will you see in labs for someone with aplastic anemia?

A

CBC revealing pancytopenia and reduced absolute number of reticulocytes, smear shows normocytic cells, with a few macrocytic cells and almost absent reticulocytes. Bone marrow aspiration, biopsy and cytogenic analysis must be performed. Bone marrow space consists mostly of fat cells and marrow stroma- decrease in all elements.Marrow-malignant cells or fibrosis are not present. Residual hematopoietic cells are morphologically normal. Rule out myelodysplastic syndrome- the precursor to leukemia.

207
Q

What will you see in labs for someone with aplastic anemia?

A

CBC revealing pancytopenia and reduced absolute number of reticulocytes, smear shows normocytic cells, with a few macrocytic cells and almost absent reticulocytes. Bone marrow aspiration, biopsy and cytogenic analysis must be performed. Bone marrow space consists mostly of fat cells and marrow stroma- decrease in all elements.Marrow-malignant cells or fibrosis are not present. Residual hematopoietic cells are morphologically normal. Rule out myelodysplastic syndrome- the precursor to leukemia.

208
Q

What will you see in someone with moderate aplastic anemia?

A

bone marrow cellularity < 30%, absence of severe pancytopenia, depression of at least 2 of the 3 blood elements

209
Q

What will you see in someone with severe aplastic anemia?

A

Bone marrow cellularity <20

210
Q

What will you see in someone with very severe aplastic anemia?

A

All criteria is met in moderate and severe aplastic anemia, ANC <200

211
Q

In who do we induce very severe aplastic anemia in?

A

Bone marrow transplant patients. We want to kill all of the cells. They are “naked” to the environment and most at risk for infection.

212
Q

What are some differential diagnoses for aplastic anemia?

A

Pancytopenia without spleenomegaly can be seen in: Acute leukemias, myelodysplastic syndrome (MDS), Fibrosis or tumor, Severe megaloblastic anemia, Paroxysmal nocturnal hemoglobinuria (PNH), Overwhelming HIV infection.
Examination of bone marrow , biopsy and smear should distinguish these as rule outs.

213
Q

What is the prognosis for aplastic anemia?

A

Depends on the severity of pancytopenia and patient age, unless severe and very severe cases are successfully treated 70% will be dead in 1 year.
Increased age causes increased mortality in all classes of disease mainly from bleeding.

214
Q

How do you treat aplastic anemia?

A 
Withdrawal of offending agent, supportive care – fluids, antibiotics, definitive therapy- HCT, immunosuppressive agents. Depends on age , under 45- HCT over 45- immunosuppression
Antithymocyte globulin (ATG) is immunotherapy , solu medrol,  cyclosporine. Transfusions should be minimized. Place a central line for ease of blood draws and therapy. Add G-CSF for neutropenia.
Mycophentolate did not improve survival. High dose cyclophosphamide without stem cell infusion.
215
Q

How do you treat aplastic anemia?

A 
Withdrawal of offending agent, supportive care – fluids, antibiotics, definitive therapy- HCT, immunosuppressive agents. Depends on age , under 45- HCT over 45- immunosuppression
Antithymocyte globulin (ATG) is immunotherapy , solu medrol,  cyclosporine. Transfusions should be minimized.
216
Q

Is B12 and folate deficiency anemia microcytic, normocytic, or macrocytic?

A

Macrocytic, this kind of anemia is associated with liver disease. The enlarged red blood cells are sometimes the only sign a patient is experiencing when they’re deficient in B12 and folate. Vitamin B!2 and folate are 2 important nutrients for the health of the nervous system. This condition can also be a side effect of chemotherapy drugs, and is a common consequence of heavy alcohol use.

217
Q

Is B12 and folate deficiency anemia microcytic, normocytic, or macrocytic?

A

Macrocytic, this kind of anemia is associated with liver disease. The enlarged red blood cells are sometimes the only sign a patient is experiencing when they’re deficient in B12 and folate. Vitamin B!2 and folate are 2 important nutrients for the health of the nervous system.

218
Q

Macrocytic anemia is a sign that the RBCs are not ___________________

A

maturing properly…cells lose their nucleus and decrease in size during the normal growth process…. when this doesn’t happen it is a sign of B12 and folate deficiency anemia

219
Q

Macrocytic anemia is a sign that the RBCs are not ___________________

A

maturing properly… this is a sign of B12 and folate deficiency anemia

220
Q

normal RBC is shaped like a biconcave disc

without the necessary nutrients, cells will not attain this shape.. this happens in what kind of anemia?

A

B12 and folate deficiency anemia

221
Q

How is B12 absorbed in the body?

A

vitamin is absorbed in the small intestine with the help of intrinsic factor, produced in the stomach lining

222
Q

What needs to be present in B12 absorption?

A

If intrinsic factor is not present, Vitamin B12 cannot be absorbed. This condition is known as pernicious anemia, and is treated with B12 injections.

223
Q

What needs to be present in B12 absorption?

A

If intrinsic factor is not present, Vitamin B12 cannot be absorbed. This condition is known as pernicious anemia, and is treated with B12 injections. Alcohol interferes with the absorption of B12, and chronic heavy drinkers are at increased risk for macrocytic anemia.

224
Q

What inerferes with the absorption of B12?

A

Alcohol interferes with the absorption of B12, and chronic heavy drinkers are at increased risk for macrocytic anemia.

225
Q

In what foods can you find folate? Should you cook these foods? How is folate absorbed in the body?

A

Folate is abundant in green leafy vegetables, liver and mushrooms. Food must be cooked to make folate available, but too much cooking destroys this vitamin. Absorption of folate takes place in the small intestine.

226
Q

What are the 4 main reasons for folate deficiency?

A

lack of folate in the diet, problems absorbing folate, difficulty utilizing the vitamin, increased requirement

227
Q

What disease makes it harder to absorb folate? What other things prevent absorption?

A

Patients with celiac disease have trouble absorbing folate because this disease damages the cells where absorption takes place.
It’s also important to note that several drugs interfere with absorption, including birth control pills. Alcohol also interferes with the use of folate.

228
Q

How will someone with folate deficiency present? (signs, symptoms, labs)

A

Severe anemia with macrocytic cells with or without neurologic disturbances. Hyper segmented neutrophils on blood smear. Pancytopenia of uncertain cause. Unexplained neurologic symptoms- dementia, weakness, sensory ataxia, parasthesias.

229
Q

How will someone with B12 and folate deficiency present? (signs, symptoms, labs)

A

Severe anemia with macrocytic cells with or without neurologic disturbances. Hyper segmented neutrophils on blood smear. Pancytopenia of uncertain cause. Unexplained neurologic symptoms- dementia, weakness, sensory ataxia, parasthesias.

230
Q

What is it called when someone is deficient in both B12 and folate?

A

Deficiency of both causes megaloblastic anemia, but only lack of B12 causes neurologic changes

231
Q

What are the symptoms of pernicious anemia?

A

Classic clinical picture of PA is a prematurely grey, Northern European person with lemon colored skin, mentally sluggish with a shiny tongue and a shuffling broad base gait. Subtle presentation is
hard to characterize, neuropsychiatric problems,
iron deficiency anemia or thalessemia.
Neuropathy affects the legs more than arms. Loss of vibration and position sense.

232
Q

What are the symptoms of pernicious anemia?

A

Classic clinical picture of PA is a prematurely grey, Northern European person with lemon colored skin, mentally sluggish with a shiny tongue and a shuffling broad base gait. Subtle presentation is
hard to characterize, neuropsychiatric problems,
iron deficiency anemia or thalessemia.
Neuropathy affects the legs more than arms.

233
Q

Symptoms of folate deficiency?

A

Onset of folate deficiency can take 4-5 months (pregnancy). Symptoms are associated with anemia.

234
Q

What special populations are commonly B12 and folate deficient?

A

Older adults – less absorption, alcoholics- cannot absorb- Wernicke’s encephalopathy, malnourished
vegans, bariatric surgery, pregnant and breast feeding women

235
Q

What labs should you do for suspected B12 and folate deficiency?

A

Measure serum cobalamin , RBC and folate levels – before any meals, metabolic intermediates
(Methylmalonate (MMA) and homocysteine accumulate in deficiency state). Antibodies to intrinsic factor which would diagnose pernicious anemia. Schilling test- radioactive cobalamin is traced. Metabolite testing (MMA) should be reserved for those with borderline levels.
Bone marrow examination is unnecessary.

236
Q

What labs should you do for suspected B12 and folate deficiency?

A

Measure serum cobalamin , RBC and folate levels – before any meals, metabolic intermediates
(Methylmalonate (MMA) and homocysteine accumulate in deficiency state). Antibodies to intrinsic factor which would diagnose pernicious anemia. Schilling test- radioactive cobalamin is traced.

237
Q

How do you treat folic acid deficiency?

A

Folic acid 1mg orally once a day for one to four months, until labs improve. Vitamin B12 deficiency must be ruled out before starting treatment since folate can mask megaloblastic anemia. Hematologic abnormalities can be partially reversed but neurologic symptoms will progress. When in doubt give both drugs until test results come back. Maintenance therapy is used for congenital abnormalities. General use of high dose folic acid without an indication is not recommended, especially at higher doses.
It can be used with chemotherapy that are folate reductase inhibitors. Cholestyramine binds it in the stomach- space doses.

238
Q

How do you treat folic acid deficiency?

A

Folic acid 1mg orally once a day for one to four months, until labs improve. Vitamin B12 deficiency must be ruled out before starting treatment since folate can mask megaloblastic anemia. Hematologic abnormalities can be partially reversed but neurologic symptoms will progress.
When in doubt give both drugs until test results come back.

239
Q

glucose 6 phosphate dehydrogenase deficiency is linked to which sex chromosome?

A

X, so it affects males more because they can show signs and symptoms

240
Q

glucose 6 phosphate dehydrogenase deficiency is linked to which sex chromosome?

A

X

241
Q

What will you see in someone with glucose 6 phosphate dehydrogenase deficiency?

A

Some are unaffected, some have episodic anemia while others have chronic hemolysis. Acute hemolytic anemia, Favism- hemolytic response to eating broad beans,congenital nonspherocytic hemolytic anemia, neonatal hyperbilirubinemia.
There may be no symptoms except shorter life span of RBC until a trigger causes hemolysis.
2 to 4 days after exposure you see sudden jaundice, pallor, dark urine with or without back pain.

242
Q

How many people world wide are affected with glucose 6 phosphate dehydrogenase deficiency?

A

200-400 million people world wide

243
Q

What is the most common trigger of hemolysis in glucose 6 phosphate dehydrogenase deficiency?

A

infection, DKA can also trigger it

244
Q

Neonatal presentation is different from Rh factor (an inherited trait that refers to a specific protein found on the surface of red blood cells. If your blood has the protein, you’re Rh positive) because………

A

Hyperbilirubinemia is not present at birth in G6PD and there is more jaundice than anemia

245
Q

Neonatal presentation is different from Rh factor because………

A

Hyperbilirubinemia is not present at birth in G6PD and there is more jaundice than anemia

246
Q

What can trigger severe hemolysis in glucose 6 phosphate dehydrogenase deficiency?

A 
Exposure to drugs  with a high redox potential can initiate severe hemolysis (Primaquine, sulfa drugs)
Fava beans can trigger a reaction. Severity of symptoms depends on level of enzyme deficiency.
Separated according to class 1 through 5 with class 1 being most severe.
247
Q

When should you consider G6PD? What will happen in a newborn to consider G6PD?

A

Should be considered in any nonimmune hemolytic anemia. Should be considered in any newborn with unexplained hyperbilirubinemia. Finding decreased activity of G6P. Fluorescent spot test is the simplest most reliable form. Methemoglobin reduction test is another useful tool. False negative results occur when the most severely deficient red cells have been removed by hemolysis and retesting is needed.

248
Q

What is methemoglobinemia? What is the treatment?

A

Methemoglobinemia is a disease resulting in hemoglobin that is less likely to allow o2 to diffuse to the tissues. Methylene blue is a treatment used, but G6P is needed for it to work therefore deficiency makes this treatment ineffective.
In addition, methylene blue can induce hemolysis because it has a high redox potential.

249
Q

What is the treatment for G6PD?

A

Avoid exposure to known triggers, pregnant and nursing women who are heterozygous should avoid drugs with a high redox potential, avoid fava beans, treat the underlying anemia. Spleenectomy and Vitamin E have occasionally helped.
Transfusions are only used for severe hemolysis.

250
Q

Which drugs are unsafe in G6PD?

A

Dapsone, Nitrofurantoin, Primaquine, Methylene blue, Phenazopyridine, Dimercaprol, Uricase, Toluine blue and all sulfa drugs.

251
Q

What chemicals are not safe in G6PD?

A

Aniline dyes, Napthalene ( moth balls), Henna compounds, Fava beans, Red wine, blueberries, soy and tonic water

252
Q

How will G6PD present in neonates? How do we treat them?

A

Neonatal jaundice can occur with irreversible brain damage. Phototherapy is used and in extreme cases exchange transfusions. Screening is not performed.

253
Q

When should you suspect G6PD for nonimmune hemolytic anemia?

A

especially after drug exposure, eating fava beans or infection

254
Q

How short is the life span for red blood cells in hemolytic anemia?

A

A shortened survival of red blood cells less than 100 days.

255
Q

What limits hemolytic anemia?

A

Compensatory increased erythropoietin secretion limits the anemia

256
Q

What increases in hemolytic anemia?

A

An increase in reticulocyte is seen as long as iron and nutrients are available. The normal reticulocyte count is 1-2%, here you can see 4-5%.

257
Q

In mild hemolytic anemia you will not see anemia unless….

A

In mild disease you will not see anemia unless compensatory mechanism cannot match the rate of hemolysis

258
Q

Intrinsic (intracorpuscular) causes for hemolytic anemia are usually _____________ and involve more than one RBC component

A

hereditary

259
Q

What are the intrinsic causes for hemolytic anemia?

A

Mature RBC has lost it’s nucleus, mitochondria and RNA. It is left with Hb, RBC membrane and the metabolic machinery to make ATP. Hemolysis can be due to the formation of Heinz bodies, membrane injury, unstable or missing proteins, hydration abnormalities, defective glycolysis.

260
Q

What are the extrinsic causes for hemolytic anemia?

A

Direct trauma, as in bongo drummers and march hemoglobinuria (runners’ or foot-strike hemolysis),
shear stress, (as in defective mechanical heart valves), Heat damage, (as in thermal burns)
Complement-induced lysis, (as in paroxysmal cold hemoglobinuria), osmotic lysis (following infusion of hypotonic solutions), lysis (from bacterial toxins) (eg, clostridial sepsis) Lysis (from exposure to high concentrations of copper).

261
Q

The severity and type of hemolytic anemia depend on what? Incompletely hemolyzed RBCs can become what?

A

The severity and type depend on the cells site of destruction. Incompletely hemolyzed RBCs, which have lost some of their membrane, can reform and become spherocytes or microspherocytes, while others are destroyed outright. Red cell “ghosts”, which are red cell membranes devoid of hemoglobin (Hgb), may be seen under extreme circumstances.

262
Q

What can any form of hemolysis lead to?

A

Any form of hemolysis can lead to renal damage due to the heme proteins and breakdown products.
Hematology should always be consulted since the hemolysis can suddenly turn life threatening.

263
Q

Where are severely damaged RBCs destroyed or phagocytosed?

A

Severely damaged RBC’s are destroyed in the liver or phagocytosed in the spleen

264
Q

What may you see in someone with hemolytic anemia?

A

Rapid onset of pallor and anemia, jaundice with increased indirect bilirubin concentration, history of pigmented (bilirubin) gallstones, splenomegaly,
presence of circulating spherocytic red cells (eg, autoimmune hemolytic anemia, congenital spherocytosis), other informative red cell shape changes.

265
Q

What labs will you find in someone with hemolytic anemia?

A

Increased serum lactate dehydrogenase (LDH) and indirect bilirubin concentration, reduced or absent level of serum haptoglobin, A positive direct antiglobulin test (Coombs test), increased reticulocyte percentage or absolute reticulocyte number (not specific for hemolysis), indicating the bone marrow’s response to the anemia. Abnormal smear.

266
Q

A patient may have hemolysis and blood loss and the patient’s compensatory __________ may mask the signs of __________

A

erythropoietin; anemia

267
Q

The combination of what 2 things is 90% specific for diagnosing hemolysis? What combination of 2 things is 92% sensitive for ruling out hemolysis?

A

Diagnosing hemolysis — The combination of an increased serum lactate dehydrogenase (LDH) and a reduced haptoglobin is 90 percent specific for diagnosing hemolysis, while the combination of a normal serum LDH and a serum haptoglobin >25 mg/dL is 92 percent sensitive for ruling out the presence of hemolysis.

268
Q

Hemolysis without high reticulocyte count may mean what?

A

Hemolysis without high reticulocyte may mean precursor cells may also be affected or the bodies response is not sufficient-Damaged marrow or
chemotherapy

269
Q

What is the treatment for hemolytic anemia?

A

There is no generic treatment for hemolytic anemia. Appropriate management of a patient with hemolytic anemia requires that the underlying cause be determined, and may include red cell transfusion if the anemia is severe.

270
Q

Reduced availability of Iron for __________ causes anemia

A

erythopoiesis

271
Q

Why does iron deficiency occur?

A

An imbalance between iron uptake and demand (usually increased demand). Absolute deficiency is when iron stores are absent due to malnourishment, malabsorption or blood loss. Functional deficiency is insufficient iron due to anemia of chronic disease (inflammation) or epogen use.

272
Q

What is overt blood loss?

A

Hemorrhage, hematemesis, melena, hemoptysis, menorrhagia, hematuria, blood donations.

273
Q

What is occult blood loss?

A

Usually GI, Always rule out a bleed first because this diagnosis requires immediate attention

274
Q

What are some lesser known causes of iron deficiency anemia?

A

Decreased absorption due to achlorhydria, food/med interactions, celiac disease, demands of pregnancy, infancy and early childhood, increased demand due to epoetin alpha use to produce more RBC, dialysis and chemotherapy patients

275
Q

What is the most common anemia?

A

iron deficiency anemia

276
Q

What happens to blood cells in the absence of iron? What are the symptoms?

A

In the absence of iron for hemoglobin the cells shrink and become microcytic (small) and hypochromic (pale). Reduced o2 carrying capacity causes listlessness, fatigue, pallor
In severe cases tachypnea, dyspnea and angina result

277
Q

What are the systemic manifestations of iron deficiency?

A

Behavioral and neuropsychiatric manifestations, pica (pagophagia) specifically for ice, angular stomatitis ( sores on the corners of the mouth), glossitis, esophageal webs and strictures, koilonychia (spoon nails), Beeturia (ingestion of beets leads to red urine), restless leg syndrome, (responds to iron therapy even if labs are normal),
fatigue and exercise intolerance, headache,
irritability

278
Q

What is the classic presentation for someone with iron deficiency anemia?

A

Multigravid woman in her forties presents with fatigue and heavy periods, Hb low at 8, MCV low at 75, MCH is low, blood smear shows microcytic, hypochromic cells. Serum iron low at 10. Total iron binding capacity (TIBC) is high- meaning there is plenty of ability to bind iron. Iron stores are absent in bone marrow. We typically do not see the classic presentation though.

279
Q

What could you see in someone with iron deficiency anemia that does not support their diagnosis?

A

Many have normal RBC indices and a normal blood smear. Onset is insidious and presents with symptoms of other diseases- CAD, dementia, CHF.

280
Q

What labs will you see for someone with iron deficiency anemia?

A

Ferritin level between 10-15. Microcytic, hypochromic erythrocytes. Absence of hemosiderin- an iron storing complex within cells.
Reduced RBC and reticulocyte Hb, Hb, Hct. Increased iron binding capacity. Once a diagnosis is made finding the cause is imperative. Usually seen with ESA’s.

281
Q

What are some differential diagnoses for iron deficiency anemia?

A

Is extensive when anemia is mild and red cell indices are normal. Anemia of chronic inflammation (ACI) is the most common. Alternative diagnosis followed by anemia of renal failure and endocrine disorders.
Most important differential diagnosis is ACI and thalassemia.

282
Q

How do you rule out anemia of chronic diease (ACD) and thalassemia when you think someone may have iron deficiency anemia?

A

In anemia of chronic disease (ACD) serum iron and TIBC will be low with a normal or increased ferritin. Thalassemia may have a positive family history, splenomegaly, target cells and tear drop cells on peripheral smear, normal iron and ferritin levels and normal RBC.

283
Q

How do you treat iron deficiency anemia?

A

Ferrous sulfate 325mg po three times a day. It is
cheap and effective. GI side effects decrease compliance-NVD, black stools.Therapy is usually 4 to 6 months. Liquid preparations may stain teeth.
Space doses if taking TCN, flouroquinolones, levothyroxine, vit E and cholestyramine.
Transfusions are reserved for the hemodynamically unstable.

284
Q

Why do we use ferrous salts instead of ferric for iron deficiency anemia? What helps absorption of it?

A

Ferrous salts are absorbed better than ferric. Ascorbic acid increases absorption and toxicity. Iron is absorbed best on an empty stomach. Iron should not be given with antacids. Iron polysaccharide complex (Niferex) seems to be better tolerated than other iron salts.

285
Q

What are some agents available for parenteral iron?

A

Iron dextran, ferric gluconate complex (Ferrlecit), iron sucrose (Venofer), Ferumoxytol (Feraheme)

286
Q

How do people usually respond to iron supplement when they have iron deficiency anemia? When might it not work for them?

A

Pica or restless leg syndrome usually disappear immediately upon treatment. Moderate anemia will see results in 7-10 days. Failure to respond could mean: Incorrect diagnosis, non-compliance, co-morbidity, underdose or malabsorption.

287
Q

How do you distinguish iron deficiency anemia from other anemias with low reticulocyte count?

A

Decreased stimulation of RBC production in bone marrow, (Beta thalassemia, Anemia of chronic disease, Chronic renal insufficiency). Isolated decrease in RBC precursors (Red cell aplasia). Bone marrow damage, (Fibrosis Stem cell damage, infiltration with tumor/infection). Intrinsic bone marrow disease, (Myelodysplasia/sideroblastic anemia).

288
Q

What should you consider in patients with relapsed/refractory iron deficiency?

A

‘Gastrointestinal sideropenia’= celiac disease, atrophic body gastritis, h. pylori infection, and gastric bypass surgery.

289
Q

What is total iron binding capacity (TIBC)?

A

Iron travels in the bloodstream bound to transferrin. If there are more binding sites available, iron stores are low.

290
Q

What is serum ferritin used for?

A

iron is stored in the spleen, liver & bone marrow as ferritin or hemosiderin. Serum ferritin measures the free iron in the serum.

291
Q

What is serum iron used for?

A

Amount of iron in the blood. A low serum iron level and increased TIBC indicate iron deficiency.

292
Q

What happens in the body in someone with sickle cell anemia?

A

Characterized by vaso-occlusive phenomena and hemolysis. Vaso-occlusion causes pain crises and organ dysfunction leading to life long disabilities and early death. Causes a very poor quality of life.

293
Q

How is sickle cell disease acquired?

A

Homozygous genetic disease of varying severity

294
Q

What happens to hemoglobin in sickle cell disease?

A

Results from a substitution of a valine for glutamic acid as the sixth amino acid of the globulin chain that creates a tetramer of hemoglobin that is poorly soluble when deoxygenated

295
Q

What happens to red blood cells in sickle cell disease?

A

cell membrane structure changes, it adheres to endothelium better than normal blood cells, the sickle shape cannot squish through vessels

296
Q

What homozygous trait does sickle cell anemia describe? Anemia is is less severe in sickle cell when what other disease exists along with it?

A

Sickle cell anemia describes the homozygous Hb S trait. It is most severe for homozygous Hb S. Intermediate intensity for Hb SC disease. Benign for those who have the trait. When Thalassemia co-exists the anemia is less severe.

297
Q

What is the most common reason sickle cell patients seek medical attention?

A

Dactylitis- pain in hands and feet that is excruciating. Waxing and waning of acute pain on top of chronic pain.

298
Q

What happens to the spleen in sickle cell?

A

Spleen sequestration- acute painful enlargement of the spleen due to intrasplenic trapping of RBC’s.
An emergency- hypovolemic shock can occur. Hemoglobin levels will drop by 2.

299
Q

Pain may be precipitated by what events in sickle cell?

A

May be precipitated by weather, wind, low barometric pressure, pollutants, menses, illness, dehydration, stress, alcohol consumption and infection. Pain episodes last 2-7 days typically.

300
Q

What is sickle cell therapy affected by?

A

Therapy is affected by opioid clearance, hyperalgesia and pseudo addiction

301
Q

What could occur in someone with sickle cell as they grow?

A

24% of patients will have an overt stroke by age 45, 25% of children will have ischemic lesions that will impair cognitive function. As they age there is greater risk of neurocognitive decline and intracranial hemorrhage. Epilepsy is 2-3 times more common. Psychosocial issues and depression. Delayed puberty and growth development are seen. Reduced or absent spleen function due to splenic infarction.

302
Q

How can you save someone with multiorgan failure with sickle cell disease?

A

May be reversed by prompt reverse transfusion therapy

303
Q

What infections are common in people with sickle cell disease?

A

Especially encapsulated organisms like Strep. Pneumoniae and H. influenza. Parvovirus and H1N1 are more virulent in this population. Bacteremia can occur by Strep pneumo that has 20-50% mortality. Occurs less often in adults.
Prophylactic antibiotics are given.

304
Q

What are other complications you may see in sickle cell?

A

Meningitis (infants and children more commonly), bacterial pneumonia. acute chest syndrome, osteomyelitis, vasooclusion causes leg ulcers, priapism, pulmonary complications are the most common causes of death. Renal failure, retinopathy, MI, increased cardiac output (heart failure).

305
Q

What hebatobiliary problems will you see in someone with sickle cell?

A

Acute hepatic ischemia, benign cholestasis, hepatic sequestration crisis, transfusional iron overload, acute and chronic cholelithiasis secondary to pigmented gallstones, acute and chronic liver disease secondary to hepatitis C virus infection (HCV) complicating blood transfusion, drug toxicity.

306
Q

What labs may you see in someone with sickle cell?

A

Anemia with reticulocytosis, hyperbilirubinemia, elevated LDH, low haptoglobin, blood smear reveals sickled cells that are normochromic and normocytic.

307
Q

How do you treat someone with sickle cell?

A

preventative care with hematologist and internist, prophylactic antibiotics (penicillin at least until age 5), vaccines (pneumovax and flu), transfusion for stroke risk. Hydroxyurea is used to reduce Hb polymerization.Chronic transfusion is the treatment for CNS injury like a stroke. Morphine and methadone for pain.

308
Q

What vitamin supplements should you give to someone with sickle cell?

A

Folic acid, Vit D, MVI without iron and calcium

309
Q

How do you prevent possible problems in someone with sickle cell?

A

HCT has been used in patients under 16 years of age and is the only cure. Patient education of the signs and symptoms of complications. Fever needs to be treated as a medical emergency. Genetic counseling. Avoid anything that stimulates bone marrow. Maintain adequate oxygen saturations. Monitor BP, retinal changes, renal function, depression, skin for ulcers and pain control.

310
Q

What is the prognosis for someone with sickle cell?

A

Has improved from a fatal childhood illness to a chronic disease with progressive deterioration for those with access to comprehensive care
The median age at death for those with homozygous sickle Hgb (HgbSS) was 42 years for men and 48 years for women.The median age at death for those with HgbSC disease was 60 years for men and 68 years for women. (this was before hydroxyurea was used).

311
Q

What do you do to secure the diagnosis of sickle cell?

A

Is made with DNA testing, screening helps to identify while in utero, heel blood is used to test for sickle cell and thalassemia at birth, sickled cells are seen on blood smear. It iIs diagnosed in childhood.

312
Q

What happens to hemoglobin in thalassemia?

A

A genetic disorder where a there is a reduction in one of the globin chains of hemoglobin.
May be a problem of the alpha or beta portion of the globin. It is a homozygous disease. Defect can be alpha or beta chain with beta occurring more commonly, (Beta chains are reduced but alpha stays the same and therefore accumulate in RBC and shortens its life cycle, Hypochromic microcytic). Confirmed by electrophoresis of Hb.
Minor disease can be misdiagnosed as iron deficiency anemia.

313
Q

What are the symptoms of thalassemia?

A

May have minor or major manifestations.

Usually apparent in infancy – pale, listless, poor appetite, slow growth, jaundice. Enlarged heart, spleen and liver.

314
Q

What are the symptoms of thalassemia minor?

A

Thalassemia minor can be asymptomatic. If the patient is heterozygous the anemia is mild and therefore termed thalassemia minor.

315
Q

If someone is homozygous for thalassemia what will they have?

A

If the patient is homozygous the anemia is chronic and severe and is termed thalassemia major and it is usually fatal in childhood

316
Q

What are beta thalassemia major symptoms?

A

Symptoms start at 6 months of agem diagnosis occurs 6-12 mos-Chronic anemia, hemolysis –profound, noxious effects of massive ineffective erythropoiesis, pallor, irritability
growth retardation, hepatosplenomegaly – abdominal swelling, jaundice. Major symptoms: Skeletal changes are profound (chipmunk face, ribs and bones become box-like), Liver and gall bladder problems (hepatospenomegaly, gall stones) and kidneys are enlarged and urine is dark, Endocrine symptoms (growth problems hypothyroidism and gonadism), Cardiac problems (heart failure, arrhhythmias), aplastic crisis (parvovirus), and pain!

317
Q

What labs will you see in someone with thalassemia?

A

Profound hypochromic microcytic anemia, Bizarre red cell morphology, WBC strikingly high, reticulocyte level low, PLT normal, Iron levels are high, Ferritin is high, Serum is icteric (yellow), bone marrow shows erythroid hyperplasia.

318
Q

prognosis for thalassemia?

A

80% of children will die in the first 5 years-

Manifested by organ damage from iron overload

319
Q

How do you treat thalassemia?

A

Spleenectomy , Iron chelation, Folic acid supplement, Hypertransfusion.

320
Q

What is the only thalassemia you will treat? What happens with the other forms of thalassemia?

A

Thalassemia A major= still birth, major, incompatible with life.Thalassemia A minor= only a carrier, you have no symptoms.Thalassemia B major= the only ones you will treat. Thalassemia B minor=only carriers, no symptoms.

321
Q

Where are myeloid neoplasms derived from? What are they restricted to developing into?

A

Myeloid neoplasms- derived from bone marrow progenitors that are restricted to developing into erythrocytes, granulocytes (neutrophils, basophils, and eosinophils), monocytes, or megakaryocytes.

322
Q

What does myeloid mean?

A

granulocyte precursor

323
Q

What are the three classes of myeloid neoplasms?

A

Acute myeloid leukemias, myeloproliferative neoplasms, and myelodysplastic syndromes.

324
Q

How aggressive is acute myeloid leukemia? What percentage of myeloid blasts in the blood or bone marrow is considered AML?

A

AML is very aggressive and requires immediate attention-Defined as >20% myeloid blasts in the bone marrow or peripheral blood.

325
Q

Myeloid sarcoma is a type of acute myeloid leukemia. What is it?

A

It is a solid tumor composed of immature white blood cells called myeloblasts. A chloroma is an extramedullary manifestation of acute myeloid leukemia; in other words, it is a solid collection of leukemic cells occurring outside of the bone marrow.

326
Q

What could occur alongside acute myeloid leukemia?

A

AML with myelodysplasia-related changes (Myelodysplasia=in which the normal process of making mature blood cells (red blood cells, white blood cells, and platelets) – known as hematopoiesis – is impaired).

327
Q

What are therapy-related myeloid neoplasms? What therapies cause them?

A

heterogeneous group of clonal hematopoietic stem cell disorders that are directly related to previous cytotoxic chemotherapy and/or radiation therapy.

328
Q

What are two predominant types of therapy-related neoplasms?

A

Two predominant and clinically significant types of therapy-related myeloid neoplasms (t-MN) have been defined, those arising after treatment with alkylating chemotherapy and/or radiation therapy and those arising after therapy with topoisomerase II inhibitors.

329
Q

What occurs to the chromosomes in acute myeloid leukemia with recurrent genetic abnormalities?

A

AML with translocations between chromosome 8 and 21, AML with inversions in chromosome 16,
AML with translocations between chromosome 15 and 17 and AML with translocations in chromosomes 9 and 11. There are also myeloid proliferations related to down syndrome.

330
Q

What are blastic plasmacytoid dendritic cell neoplasms in acute myeloid leukemia? How common is it? How aggressive is it?

A

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination.

331
Q

hemocytoblast –> proerythroblast –> polychromatic erythroblast –> ?

A

erythrocytes

332
Q

hemocytoblast –> myeloblast –> progranulocyte –> ?

A

basophils, eosinophils and neutrophils (these are all granulocytes, a type of leukocyte)

333
Q

hemocytoblast –> lymphoblast –> ?

A

lymphocyte (agranulocyte, a type of leukocyte)

334
Q

hemocytoblast –> monoblast –> ?

A

monocyte (agranulocyte, a type of leukocyte)

335
Q

hemocytoblast –> megakaryoblast –> megakaryocyte –> ?

A

thrombocyte

336
Q

A myeloproliferative neoplasm type is chronic myeloid leukemia. What is overproliferating?

A

Chronic myeloid leukemia (CML) — CML demonstrates overproliferation of the granulocytic lineage at all stages of maturation.

337
Q

What gene is associated with chronic myeloid leukemia? Does i affect treatment?

A

It is always associated with the presence of a BCR-ABL fusion gene, which is usually created by a reciprocal translocation that results in the formation of the Philadelphia chromosome. It is reported as Ph chromosome +. It does affect treatment.

338
Q

What is a myelodysplastic syndrome?

A

It refers to disorders that exhibit dysplasia and ineffective blood cell production with a variable risk of transformation to acute leukemia. It is a precursor to leukemia.

339
Q

In myelodysplastic syndrome does bone marrow cellularity increase or decrease? Do the blasts in the bone marrow increase or decrease?

A

Bone marrow cellularity is often increased, but may be normocellular or hypocellular. The percentage of blasts in the bone marrow is normal or increased, but less than 20 percent.

340
Q

What happens in myelodysplastic syndrome?

A

Maturation is present, but dysplasia is noted in one or more myeloid lineages. Hematopoiesis is ineffective and results in cytopenias.

341
Q

Lymphoid neoplasms are those derived from cells that normally develop into what?

A 
T lymphocytes (cytotoxic T lymphocytes, helper T lymphocytes, or regulatory T lymphocytes) and 
B lymphocytes (lymphocytes or plasma cells). In general, the lymphoid neoplasms are divided into neoplasms derived from lymphoid precursors and neoplasms of mature lymphocytes and plasma cells.
342
Q

It is now appreciated that any “lymphoma” can present with or evolve to a leukemic picture, and any “leukemia” can occasionally present with a mass lesion. How did we used to classify these malignancies?

A

Historically, lymphoid neoplasms that present with bone marrow and blood involvement (leukemia) have been segregated from those that present as a mass (lymphoma).

343
Q

In the WHO classification, the diagnosis of the various lymphoid neoplasms depends on what?

A

not on the anatomic location of tumor cells, but rather on the cell of origin of the tumor.

344
Q

What are the three different lymphoid neoplasm groups?

A

Indolent, aggressive and highly aggressive. These are all uncommon.

345
Q

What amount of time is indolent lymphoid neoplasms measured in? What percentage is non-hodgkins lymphoma?

A

Survival of untreated indolent lymphoid neoplasms is generally measured in years. Indolent lymphomas represent 35 to 40 percent of the non-Hodgkin lymphomas (NHLs).

346
Q

What amount of time is aggressive lymphoid neoplasms measured in? What percentage of NHLs are aggressive?

A

Survival of untreated aggressive lymphoid neoplasms is usually measured in months. About half of the NHLs are aggressive.

347
Q

What amount of time is highly aggressive lymphoid neoplasms measured in? What kind of cells does it arise from?

A

Survival of untreated highly aggressive lymphoid neoplasms is measured in weeks.The highly aggressive lymphomas can arise from B cells or T cells.

348
Q

The highly aggressive precursor lymphoid neoplasms are those that are comprised of immature B or T cells. What are the 2 main categories?

A

The precursor B lymphoblastic leukemia/lymphomas are neoplastic disorders of immature lymphoblasts committed to the B cell lineage. The precursor T lymphoblastic leukemia/lymphomas are neoplastic disorders of immature lymphoblasts committed to the T cell lineage that may arise within the thymus or the bone marrow.

349
Q

What is the most common leukemia in Western countries?

A

A mature B cell neoplasm, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), — is the most common leukemia in Western countries, accounting for approximately 30 percent of all leukemias in the United States.

350
Q

What is lymphoplasmacytic lymphoma (LPL) waldenstrom macroglobulinemia?

A

A type of b cell neoplasm, chronic lymphocytic leukemia/small lymphocytic lymphoma

351
Q

Mantle cell lymphoma (MCL)- how common is it?

A

— comprises about 7 percent of adult NHLs in the United States and Europe.

352
Q

What is the second most common lymphoma in the U.S.? How does it present?

A

Follicular lymphoma (FL) — is the second most common lymphoma in the United States and Western Europe. Patients usually present with painless peripheral adenopathy.

353
Q

What kind of cells does hodgkin’s lymphoma arise from? What is the buzz word for the smaller amount of neoplastic cells present?

A

Hodgkin’s lymphoma- formerly called Hodgkin’s disease, arises from germinal center or post-germinal center B cells. HL has a unique cellular composition, containing a minority of neoplastic cells (Reed-Sternberg cells and their variants) in an inflammatory background.

354
Q

What is the typical prognosis for hodgkin lymphoma?

A

Hodgkin lymphoma, although a lymphoid malignancy, is pathologically and clinically distinct from the other lymphoid neoplasms and is considered a distinct entity with a generally excellent prognosis

355
Q

What virus is sometimes associated with hodgkin lymphoma?

A

A significant minority of cases of classical HL are EBV-associated, particularly the lymphocyte depleted and mixed cellularity types

356
Q

What sub-type of HL is never associated with EBV?

A

nodular lymphocyte-predominant HL is never EBV-associated

357
Q

What are the different types of cutaneous T cell lymphomas?

A

There are numerous cutaneous T cell lymphomas. Mycosis fungoides (MF) is the most common of these. Sézary syndrome is an erythrodermic, leukemic variant of MF.

358
Q

Leukemia presents with a tumor placed where?

A

Refers to a neoplasm of hematopoietic tissue as opposed to a solid tumor

359
Q

How do leukemic cells reach the body’s organs?

A

Leukemic cells infiltrate the bone marrow and lymphoid tissue and spill into the bloodstream and infiltrate the bodies organs.

360
Q

Are leukemic cells mature or immature?

A

The cells may be mature or immature.

361
Q

What will WBC count look like in someone with leukemia?

A

The overproduction of WBC’s can be seen on CBC, unless disease is confined to the bone marrow.

362
Q

How is leukemia classified?

A

Classified according to cell type and maturity

363
Q

Which cells usually cause leukemia?

A

Any cell can cause leukemia, but it is usually granulocytic, monocytic or lymphocytic, (there are subtypes based on cell membrane characteristics and genotype).

364
Q

In leukemia, If the cells are mostly primitive it is termed _____. If the cells are mature it is termed _____.

A

acute; chronic

365
Q

In chronic granulocytic leukemia most of the circulating cells are what types?

A

In chronic granulocytic leukemia most of the circulating cells are maturing granulocytes and neutrophils but there are few primitive cells.

366
Q

In chronic lymphocytic leukemia what does the WBC count look like?

A

In most cases the WBC will be profoundly elevated (30).

367
Q

What is aleukemic leukemia?

A

Aleukemic leukemia is when the disease is confined to the bone marrow and blood counts appear normal.

368
Q

Why does anemia occur in leukemia?

A

Because the leukemic cells crowd out the bone marrow there is anemia due to ineffective erythopoiesis, infection and bleeding from thrombocytopenia.

369
Q

Where can leukemic cells spread to?

A

Leukemic cells make it to the bone, liver, spleen and lymph nodes.

370
Q

How quickly does chronic leukemia progress? How well can you control it?

A

Chronic disease proceeds at a slow pace and can be well controlled for long periods of time.

371
Q

How quickly does acute leukemia progress?

A

rapidly progressive

372
Q

Arrest of disease progression from treatment with chemotherapy is called what?

A

remission

373
Q

In many cases after leukemia remission, what happens?

A

the disease relapses and proves fatal

374
Q

What do you use to treat philadelphia + cancers?

A

Philadelphia + cancers can be successfully treated with tyrosine kinase inhibitors.

375
Q

How does an autologous cell bone marrow transplant work?

A

Your own cells are collected during remission for you to use later if you need them. It can be used if there is no match. Patients cells are collected and treated with leukemia killing antibodies.

376
Q

After an autologous cell bone marrow transplant, what are the chances of a graft-versus-host disease or a relapse?

A

Less chance of GVHD more chance of relapse

377
Q

With a allogeneic bone marrow transplant, (donor with similar HLA), what are the chances of a graft-versus-host disease or a relapse?

A

More of a chance of GVHD less chance of relapse. All cells must be destroyed by chemotherapy.

378
Q

What happens in a graft versus host disease?

A

Graft vs host disease (GVHD)- the donor lymphocytes attack the hosts cells (transplant rejects the patient)

379
Q

What are the complications associated with a bone marrow transplant?

A

Transplants are not always successful. Infection

and a relapse could occur if all cells were not destroyed.

380
Q

What happens during a bone marrow transplant?

A

New cells transfused and “move in” to the marrow

381
Q

In which type of bone marrow transplant, autologous or autogenic, is immunosuppression needed?

A

Immunosuppression is necessary only in allogenic

382
Q

Myelodysplastic syndrome is the precursor for what? How long will it take to appear?

A

leukemia; may take several months to several years for leukemia to appear

383
Q

What will you see on a CBC for someone with myelodysplastic syndrome?

A

Moderate leukopenia, thrombocytopenia, anemia

384
Q

What is the treatment for someone with myelodysplastic syndrome?

A

No specific treatment, watch and wait.

385
Q

In multiple myeloma, the neoplasm of plasma cells is generally confined to where?

A

bone marrow

386
Q

What are some bad symptoms of multiple myeloma?

A

May form discrete tumors that weaken the bone and cause spontaneous fractures. Lytic lesions, “CRAB” (calcium, renal, anemia, bone)

387
Q

What levels do you monitor in someone with multiple myeloma?

A

Ca, Scr, and CBC

388
Q

In multiple myeloma, neoplastic cells cause a large increase in what? What does that increase?

A

Neoplastic cells cause large amounts of blood protein to increase and therefore blood viscosity.

389
Q

What is the problem with leukemia

A

Protein is usually IgG, proteins can accumulate in tissues and organs and block function. Protein is usually IgG. Proteins can accumulate in tissues and organs and block function.

390
Q

Is multiple myeloma a chronic disease?

A

This is a chronic disease that will wax and wane

391
Q

How do you treat multiple myeloma?

A

BMT- improves survival but does not cure the disease. Thalidomide (remember toxicities)
suppresses blood vessel proliferation that support myeloma cells. bortezomib inj. Watch if dose is SC or IV localized radiation to affected bone.

392
Q

What is premyeloma?

A

asymptomatic period before myeloma presents, lasts years, Happens in adults over 65, patients may not live long enough for the disease to appear

393
Q

What is premyeloma?

A

asymptomatic period before myeloma presents, it happens in adults over 65, some patients may not live long enough for the disease to appear

394
Q

How often do people die from malignant neoplasms?

A

Malignant neoplasms are the leading cause of disability and death. Cancer is second only to heart disease for US cause of death (23%). Lung in men and breast in women are the most common solid tumors. Survival depends on early diagnosis and treatment before the disease has spread (metastasis).

395
Q

What are the survival rates for multiple myeloma when it comes to the thyroid? Pancreas?

A

Curability is assessed in terms of 5 year survival rates 95% for thyroid cancer, 5% for pancreatic cancer

396
Q

Some cancers are prone to late recurrence, which cancers are these?

A

Breast and melanoma, this means the disease “hides” and is kept at bay until the immune system cannot contain it anymore

397
Q

The diagnostic evaluation of a patient suspected of having chronic lymphocytic anemia leukemia should include what?

A

A complete blood count with differential
examination of the peripheral smear
immunophenotypic analysis of the circulating lymphocytes. Bone marrow aspirate and biopsy and lymph node biopsy are not among the required elements of the diagnostic work-up. Chromosomal changes seen in CLL are also not diagnostic features of the disease

398
Q

What happens to the lymphocytes in chronic lymphocytic anemia?

A

chronic lymphoproliferative disorder (lymphoid neoplasm) characterized by a progressive accumulation of functionally incompetent lymphocytes, which are monoclonal in origin.

399
Q

What is chronic lymphocytic anemia considered to be identical to?

A

The disorder is considered to be identical (i.e., one disease at different stages) to the mature B cell neoplasm small lymphocytic lymphoma

400
Q

The peripheral blood smear of patients with CLL demonstrates ______________?

A

lymphocytosis

401
Q

What are smudge cells?

A

The smear of chonic lymphocytic anemia. It often contains “smudge” cells that are lymphocytes that appear to have been flattened or smudged in the process of being spread on the glass slide.

402
Q

What tests do you perform to confirm chronic lymphocytic anemia?

A

Immunophenotypic analysis, usually by flow cytometry, is a key component to the diagnosis of CLL

403
Q

The peripheral blood smear of patients with CLL demonstrates what?

A

lymphocytosis

404
Q

Why does chronic lymphocytic anemia associate with ‘smudge cells’?

A

The smear contains “smudge” cells that are lymphocytes that appear to have been flattened or smudged in the process of being spread on the glass slide

405
Q

What are the three immunophytes of chronic lymphocytic anemia?

A

Expression of B cell associated antigens including CD19, CD20, and CD23.

406
Q

How are the three immunophytes of chronic lymphocytic anemia individually expressed?

A

Expression of CD20 is usually weak. Expression of CD21 and CD24 can be seen, but is not required for diagnosis. There is also expression of CD5, a T cell associated antigen.

407
Q

For the evaluation and diagnosis of chronic lymphocytic anemia, what 2 criteria must be met?

A

Absolute B lymphocyte count in the peripheral blood ≥5000/microL. Demonstration of clonality of the circulating B lymphocytes by flow cytometry of the peripheral blood. Extremely low levels of SmIg. Expression of B cell associated antigens (CD19, CD20, and CD23); and expression of the T cell associated antigen CD5.

408
Q

What are the differential diagnoses for chronic lymphocytic anemia? What confirms the diagnosis?

A

Lymphocytosis rises concern over CLL as well as:

Mononucleosis, pertussis, toxoplasmosis and other neoplasms. Flow cytometry confirms the diagnosis.

409
Q

What is the treatment for chronic lymphocytic anemia?

A

Not all patients require treatment at the time of diagnosis. CLL is an extremely heterogeneous disease with certain subsets of patients having survival rates w/o treatment that is similar to the normal population. With the possible exception of allogeneic hematopoietic cell transplantation (HCT), CLL cannot be cured by current treatment options.

411
Q

Prospective, randomized trials evaluating immediate versus delayed treatment strategies have proved what about chronic lymphocytic anemia?

A

Prospective, randomized trials evaluating immediate versus delayed treatment strategies have found no improvement in long-term survival with early treatment. Spontaneous regression of variable duration is a rare occurrence.

412
Q

In chronic lymphocytic anemia, what is the treatment for when it is early and asymptomatic?

A

Asymptomatic early disease
Watch and wait. No proven benefit for early treatment although this waiting period may be emotionally stressful for the patient

Hematology (1 decks)

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