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First Aid Chs Part 2 > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

What is anisocytosis?

A

RBCs of varying size

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2
Q

What is poikilocytosis?

A

RBCs of varying shapes

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3
Q

<p>What is contained in alpha/delta/lambda granules of platelets?</p>

A

<p>alpha granules - clotting factors vWF, factor V; delta granules - ADP/ATP, serotonin, histamine; lambda granules - hydrolytic enzymes (anti-coagulant)</p>

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4
Q

<p>vWF binds to what platelet receptor?</p>

A

<p>GpIb (deficiency = Bernard-Soulier syndrome)</p>

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5
Q

Fibrinogen binds to what platelet receptor?

A

GpIIb/IIIa (Deficiency = Glanzmann’s thrommasthenia)

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6
Q

CD14 is a cell surface marker for..

A

Macrophages

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7
Q

Eosinophils fight helminths using what protein?

A

Major basic protein

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8
Q

CD19 and CD20 are cell surface markers for…

A

B cells

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9
Q

What role does ADP play in the clotting cascade?

A

ADP induces GpIIb/IIIa receptor expression at platelet surface, allowing platelet aggregation.

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10
Q

What is primary hemostasis?

A

Formation of a platelet plug - mediated by interaction of the platelet and a vessel wall.

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11
Q

What is secondary hemostasis?

A

Stabilization of the platelet plug by products of the coagulation cascade.

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12
Q

What is the first step in primary hemostasis?

A

Transient vasoconstriction of blood vessels. Mediated by endothelin and neural input.

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13
Q

Where does vWF come from (2)?

A
  1. The platelet itself 2. Endothelial cells (Weibel-Palade body)
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14
Q

What two important things are contained in the Weibel-Palade body?

A
  1. P-selectin 2. vWF
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15
Q

What are the 4 steps of primary hemostasis?

A
  1. Transient vasoconstriction 2. Adhesion of platelets (vWF/GpIb) 3. Release of platelet contents (ADP and TXA2) 4. Platelet aggregation (fibrinogen/ GpIIb/IIIa)
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16
Q

What molecule do platelets use to cross-link?

A

Fibrinogen

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17
Q

TXA2 is a derivative of…

A

Platelet cyclooxygenase

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18
Q

What is the size of petechiae, purpura, and ecchymoses?

A

Petechiae - pinpoint bleeding Purpura - 3 mm or greater Ecchymoses - greater than 1 cm

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19
Q

<p>Anti-Gp IIb/IIIa antibodies</p>

A

<p>ITP</p>

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20
Q

<p>What is the cause of ITP?</p>

A

<p>IgG autoantibodies against GpIIb/IIIa receptors. Antibody bound platelets are consumed by splenic macrophages, resulting in thrombocytopenia. Decreased platelet survival </p>

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21
Q

What are the Platelet count, PT/PTT, Bone marrow biopsy in ITP?

A

Platelet count is low; PT/PTT is normal; Bone marrow biopsy shows increased megakaryocytes (trying to make more platelets)

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22
Q

What 2 effects does splenectomy have on patients with ITP?

A

Eliminates the primary source of antibody and site of platelet destruction.

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23
Q

What two disorders can produce a microangiopathic hemolytic anemia and what is the classic cell seen on peripheral smear in these disorders?

A

TTP/HUS. Will see schistocytes (helmet cells).

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24
Q

How do schistocytes form in TTP?

A

Uncleaved vWF multimers (normally cleaved by ADAMTS13) lead to abnormal platelet adhesion, resulting in microthrombi that shear RBCs.

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25
Q

What enzyme is deficient in TTP and what is its normal function?

A

ADAMTS13 - normally degrades multimers of vWF.

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26
Q

ADAMTS-13 function and associated pathology.

A

Function - degrade vWF multimers. Pathology - ADAMTS13 is defective in TTP.

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27
Q

What is the pentad of symptoms seen in TTP?

A

FATRN : fever, anemia (microangiopathic), thrombocytopenia, renal failure, neurologic symptoms.

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28
Q

How do schistocytes form in HUS?

A

Infection with E. coli O157:H7 results in production of toxin that damages endothelial cells, resulting in platelet microthrombi, which deposit and cause shearing of RBCs.

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29
Q

What is the Platelet count, PT/PTT, Peripheral smear, bleeding time and bone marrow biopsy in HUS/TTP?

A

Thrombocytopenia, increased bleeding time, NORMAL PT/PTT, peripheral smear shows anemia with Schistocytes and bone marrow biopsy shows increased megakaryocytes

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30
Q

What is the defect in Bernard-Soulier syndrome?

A

Genetic GpIb deficiency - platelet ADHESION to vWF impaired.

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31
Q

What is seen on blood smear in Bernard-Soulier syndrome?

A

Thrombocytopenia with enlarged platelets (producing immature platelets from bone marrow)

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32
Q

What is the defect in Glanzmann thrombsthenia?

A

GpIIb/IIIa deficiency - platelet AGGREGATION is impaired

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33
Q

What pathways does PT measure? aPTT?

A

PT: Extrinsic/common; aPTT: Intrinsic/common

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34
Q

List the factors unique to the intrinsic and extrinsic pathways.

A

Intrinsic: XII, XI, IX, VIII; Extrinsic: VII

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35
Q

What is used to measure heparin effect?

A

PTT

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36
Q

What is used to measure warfarin effect?

A

PT (INR)

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37
Q

What is deficient in hemophilia A?

A

Factor VIII

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38
Q

What is deficient in hemophilia B?

A

Factor IX

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39
Q

What happens to the bleeding time, PTT and PT in hemophilia A?

A

Increased PTT, normal PT, normal bleeding time

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40
Q

What happens to the bleeding time, PTT and PT in hemophilia B?

A

Increased PTT, normal PT, normal bleeding time

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41
Q

How is hemophilia A distinguished from a patient with autoantibodies for factor VIII?

A

PTT corrects in hemophilia A when patient’s plasma is mixed with normal plasma (replace factor VIII). PTT does NOT correct in patients with VIII autoantibodies (the antibodies take out factor VIII).

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42
Q

What is the most common inherited coagulation disorder?

A

von Willenbrand disease (autosomal dominant).

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43
Q

What are the two functions of vWF?

A
  1. Bind GpIb receptors on platelets to start primary hemostasis. 2. Stabilize factor VIII in the circulation
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44
Q

What happens to the bleeding time, PTT and PT in von Willenbrand disease?

A

Increased bleeding time, Increased PTT (vWF stabilizes factor VIII, normal PT, Abnormal ristocetin test

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45
Q

What is the ristocetin test?

A

Introduction of ristocetin to normal platelets causes platets to aggregate. In patients with von Willenbrand disease, platelets won’t aggregate with ristocetin.

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46
Q

What is the treatment for von Willebrand disease, how does it work, and how is it given?

A

Intranasal desmopressin - increases vWF release from Weibel-Palade bodies of endothelium.

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47
Q

What factors require vitamin K as a cofactor?

A

II, VII, IX, X, Protein C, Protein S

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48
Q

What antibodies does the patient develop in heparin-induced thrombocytopenia?

A

IgG antibody towards heparin-platelet factor 4 complex, which activates platelets.

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49
Q

What happens to the platelet count, PT, PTT in DIC?

A

Decreased platelet count, Increased PT/PTT

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50
Q

What is the best screening test for DIC?

A

Fibrin split products (D-dimer). D-dimer is elevated in DIC.

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51
Q

A patient has symptoms of DIC but no elevated D-dimer. What should you consider?

A

A disorder of fibrinolysis (elevated urokinase after a prostatectomy, decreased alpha-2 antiplasmin in liver failure).

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52
Q

What is the antidote for t-PA and other thrombolytics?

A

Aminocaproic acid.

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53
Q

What class of drugs is used to anticoagulate patients with HIT?

A

Direct thrombin inhibitors (bivalrudin, lepirudin, argatroban, dabigatran).

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54
Q

What is given for rapid reversal of heparin toxicity?

A

Protamine sulfate (positively charged molecule binds negatively charged heparin).

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55
Q

What are lines of Zahn?

A

RBC/fibrin lines seen in thrombi formed pre-mortem.

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56
Q

What is Virchow’s triad and what does it describe?

A

Disruption in blood flow, Endothelial damage and hypercoagulable state. Virchow’s triad describes the risk for thrombosis

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57
Q

How does B12 or folate deficiency increase risk for thrombosis?

A

Increased homocysteine levels in B12 and folate deficiency increase risk for thrombosis secondary to endothelial damage.

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58
Q

What is the function of proteins C and S?

A

Protein S serves as a cofactor for protein C, which inactivates factors V and VIII.

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59
Q

What will happen if you give warfarin to a patient with protein C or S deficiency and why?

A

Skin necrosis because protein C and protein S have lowest half life of the vitamin K-dependent proteins, so they decrease the fastest, resulting in a transient hypercoagulable state (unopposed factors II, VII, IX, X). Need to have heparin on board when starting warfarin.

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60
Q

Describe the pathophysiology of factor V Leiden.

A

Mutated factor V is resistant to degradation of protein C and S, leading to a hypercoagulable state.

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61
Q

Patient given heparin shows no increase in PTT. What are you thinking?

A

Anti-thrombin III deficiency, higher risk for thrombus.

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62
Q

Why does estrogen use increase risk of thrombosis?

A

Estrogen increases production of coagulation factors.

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63
Q

What is the histological hallmark of a cholesterol embolus?

A

Cholesterol clefts in the clot (white cholesterol crystals).

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64
Q

What are the signs and symptoms of a fat embolus?

A

Dyspnea (lung involvement) and petechiae on the skin overlying the chest following a broken bone.

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65
Q

Why do patients with amniotic fluid emboli get DIC?

A

Amniotic fluid has tissue thromboplastin, which activates the coagulation cascade

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66
Q

What is seen on histological examination of an amniotic fluid embolus?

A

Squamous cells and keratin debris from fetal skin.

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67
Q

What is the MCV in a microcytic anemia?

A

< 80

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68
Q

What are the 4 general causes of microcytic anemia?

A

Iron deficiency, anemia of chronic disease, sideroblastic anemia, thalassemia.

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69
Q

Where in the gut is iron absorbed?

A

Duodenum enterocytes

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70
Q

What is the function of transferrin?

A

Transportation of blood iron, delivering it to liver and bone marrow macrophages for storage.

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71
Q

What is the function of ferritin?

A

Storage of intracellular iron

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72
Q

What is the function of ferroportin?

A

Transporting iron into the blood from duodenal enterocytes

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73
Q

TIBC is a measurement of…

A

How many transferrin molecules are in the blood

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74
Q

Serum ferritin is a measurement of…

A

How much iron is present in storage in bone marrow macrophages and liver

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75
Q

Early iron deficiency anemia presents as…

A

Normocytic anemia (progresses to a microcytic, hypochromic anemia).

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76
Q

What is seen in RDW, Ferritin, TIBC, serum iron, percent saturation, and free erythrocyte protoporphyrin (FEP) in iron deficiency anemia?

A

RDW increased (varying size RBC because iron deficiency anemia is a normocytic anemia that progresses to microcytic); Decreased ferritin (decreased iron in storage); Increased TIBC (trying to use as much iron in the blood as possible). Decreased serum iron; Decreased % saturation; Increased FEP (Since heme = iron + protoporphyrin, if iron is low, protoporphyrin is still produced at normal levels, so it is high because it is not being incorporated into hemoglobin).

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77
Q

Why is free erythrocyte protoporphyrin increased in iron deficiency anemia?

A

Since heme = iron + protoporphyrin, if iron is low, protoporphyrin is still produced at normal levels, so it is high because it is not being incorporated into hemoglobin.

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78
Q

What is the triad seen in Plummer-Vinson syndrome?

A

Esophageal webs, iron deficiency anemia, glossitis. Seen in pregnant women.

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79
Q

What are the two functions of hepcidin?

A

Sequestration of iron in storage sites (“hides” iron from bacteria in anemia of chronic disease), suppression of EPO production.

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80
Q

Describe the changes seen in ferritin, TIBC, serum iron, % saturation, FEP in anemia of chronic disease.

A

Increased ferritin (hepcidin leads to increased stored iron), Decreased TIBC (want to keep iron away from bugs), decreaesd serum iron, decreased % saturation, and increased FEP

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81
Q

What causes sideroblastic anemia?

A

Defective protoporphyrin synthesis

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82
Q

What is the RLS in production of protoporphyrin?

A

Production of aminolevulinic acid by aminolevulinic acid synthase

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83
Q

What is a ringed sideroblast?

A

Iron accumulating in mitochondria in a ring around nuclei. Seen in sideroblastic anemia (iron can’t be incorporated into heme).

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84
Q

Where is iron located in ringed sideroblasts?

A

Mitochondria

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85
Q

What is the most common cause of hereditary sideroblastic anemia?

A

ALAS (aminolevulinic acid synthase) mutations - X linked.

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86
Q

ALAS (aminolevulinic acid synthase) deficiency

A

Congenital sideroblastic anemia (#1 cause).

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87
Q

What are 3 causes of acquired sideroblastic anemia?

A

Alcoholism (mitochondrial toxin); Lead poisoning (denatures ALAD and ferrochetalase); B6 deficiency (cofactor for ALAS)

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88
Q

What is the treatment for sideroblastic anemia?

A

B6 supplementation (cofactor for ALAS)

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89
Q

A patient with TB develops sideroblastic anemia. What caused this?

A

INH causes B6 deficiency, which is necessary as a cofactor for ALAS (the RLS in protoporphyrin snythesis). B6 deficiency leads to sideroblastic anemia.

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90
Q

Describe what will be seen in ferritin, TIBC, serum iron, % saturation in a patient with sideroblastic anemia.

A

Increased ferritin (bone marrow macrophages ingest excess blood iron); decreased TIBC, Increased serum iron (defective production of protoporphyrin = increased iron), Increased % saturation

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91
Q

What is the underlying pathology in thalassemia?

A

Decreased production of globin chains

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92
Q

What is the underlying cause of all forms of alpha thalassemia?

A

Alpha globin chain deletion (there are 4 copies on chromosome 16).

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93
Q

What is the consequence of deletion of 1 alpha globin gene?

A

Asymptomatic

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94
Q

What is a “cis” deletion in alpha thalassemia?

A

Deletion of two alpha globin chain genes on the same chromosome (normally have 4 copies). Increases the risk for severe thalassemia in offspring. Seen in Asian individuals.

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95
Q

<p>What is the consequence of deletion of 3 alpha globin genes?</p>

A

<p>Severe anemia. Beta chains form tetramers (HbH) that damage RBCs.</p>

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96
Q

What is hemoglobin H?

A

Beta globin tetramers seen in 3 alpha globin chain deletions.

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97
Q

<p>What is the consequence of deletion of 4 alpha globin gene?</p>

A

<p>Lethal in utero. Gamma chains form tetramers (Hb Barts) that damage RBCs. Leads to hydrops fetalis </p>

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98
Q

What is hemoglobin Barts?

A

Gamma chain tetramers seen with 4 alpha globin chain deletions (hydrops fetalis).

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99
Q

What is the underlying pathophysiology of beta thalassemia?

A

Gene mutations in beta chain

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100
Q

What is the cause of beta thalassemia minor?

A

Beta chain mutations causing underproduction of the beta chain. Usually asymptomatic, may have microcytic, hypochromic RBCs and target cells on smear.

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101
Q

Isolated increase in hemoglobin A2 on electrophoresis…

A

Beta thalassemia minor

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102
Q

What are some signs and symptoms of beta thalassemia major?

A

Expansion of hematopoiesis into marrow of skull and facial bones (causing frontal bossing and “crew cut” appearance on x-ray), hepatosplenomegaly (spleen undergoes RBC production).

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103
Q

Crew cut appearance on x-ray…

A

Beta thalassemia minor or SCA (due to marrow expansion and hematopoiesis in the skull) .

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104
Q

What is seen on electrophoresis in a patient with beta thalassemia major?

A

No HbA; Increased HbA2 (alpha 2, delta 2); Increased HbF (alpha 2, gamma 2)

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105
Q

What is HbA2 composed of?

A

Alpha 2, delta 2

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106
Q

What is HbF composed of?

A

Alpha 2, gamma 2

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107
Q

What is HbA composed of?

A

Alpha 2, beta 2

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108
Q

What is the MCV in a macrocytic anemia?

A

> 100

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109
Q

Why are the cells big in a macrocytic anemia?

A

RBCs undergo one less division because of disruption of DNA precursors.

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110
Q

Where in the gut is folate absorbed?

A

Jejunum

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111
Q

What happens to the levels of serum homocysteine and methylmalonic acid in B12 and folate deficiency?

A

B12 deficiency: both homocysteine and MMA are elevated; Folate deficiency: only homocysteine is elevated, MMA is normal.

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112
Q

Normal MMA, high homocysteine

A

Folate deficiency

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113
Q

High MMA, high homocysteine

A

B12 deficiency.

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114
Q

Why does pancreatic insufficiency cause B12 deficiency?

A

Because the pancreas produces R binder which binds and protects B12.

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115
Q

Why does Crohn disease cause B12 deficiency?

A

Because B12 is absorbed in the ileum

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116
Q

What is an infectious cause of B12 deficiency?

A

Diphyllobothrium latum tapeworm infection

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117
Q

Why is dietary deficiency of B12 rare?

A

Because the liver has lots of stores of B12.

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118
Q

What is subacute combined degeneration and what causes it?

A

Degeneration of multiple tracts of the spinal cord (DCML, LCST) due to B12 deficiency causing defective myelin synthesis.

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119
Q

What is the MCV in normocytic anemia?

A

80-100

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120
Q

What are the two general causes of normocytic anemia and how are the two distinguished?

A

Increased peripheral destruction or underproduction of RBCs. Distinguish between the two by looking at reticulocyte count. Reticulocytes will be increased with RBC destruction.

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121
Q

Why are reticulocytes blue?

A

Because of RNA in the cytoplasm

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122
Q

Why are reticulocytes falsely elevated in anemia?

A

Because they are measured as a percentage of RBCs. If RBC mass is decreased and reticulocyte count stays constant, the percentage is increased.

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123
Q

How is reticulocyte count corrected for in normocytic anemia?

A

Multiplying reticulocyte count by Hct/45.

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124
Q

Normocytic anemia with corrected reticulocyte count > 3%

A

Autoimmune anemia

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125
Q

Normocytic anemia with corrected reticulocyte count < 3%

A

Problem with bone marrow production of RBCs

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126
Q

Macrophage consumption of RBCs produces which byproducts?

A

Globin –> amino acids; Heme –> iron + protoporphyrin; Protoporphyrin –> unconjugated bilirubin (bound to albumin, goes into bile).

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127
Q

What is the function of haptoglobin?

A

Binds iron in the blood from metabolized RBCs, takes it to the spleen to be re-processed

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128
Q

What happens to haptoglobin levels in intravascular hemolysis?

A

Decreases (it binds iron to return it to make new RBC). This is the initial change with intravascular hemolysis.

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129
Q

What causes hemosiderinuria in hemolytic anemia?

A

Iron being taken up be tubular cells, which slough off after a few days.

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130
Q

What are the 3 most common mutations in hereditary spherocytosis and what is the normal function of these proteins?

A

Ankyrin, spectrin, band 3.1 proteins - normally serve as cytoskeleton membrane tethering proteins.

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131
Q

Why is RDW increased in hereditary spherocytosis?

A

Red cells differ in size because they form membrane blebs which are constantly removed by the spleen over time. Older cells have had more blebs removed and are smaller.

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132
Q

Why does the spleen enlarge in hereditary spherocytosis?

A

Work hypertrophy - the macrophages hypertropy because they consume the blebs on the spherocytic RBCs.

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133
Q

Parvovirus B19 infects…

A

Erythroid precursor cells (aplastic crisis in SCA, HS).

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134
Q

Positive osmotic fragility test…

A

Hereditary spherocytosis

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135
Q

Test used to diagnose hereditary spherocytosis

A

Osmotic fragility test

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136
Q

Tretment for hereditary spherocytosis

A

Splenectomy

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137
Q

What are Howell-Jolly bodies and why do they form?

A

Basophilic nuclear remnants found in RBCs following splenectomy. They form because the spleen functions to remove abnormal RBCs from the circulation.

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138
Q

What is the mutation in sickle cell anemia?

A

Glutamic acid –> valine at position 6 on beta chain of hemoglobin. Result is hemoglobin S.

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139
Q

Why do patients with sickle cell disease not present until a few months old?

A

Because HbF is protective for the first few months of life.

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140
Q

MOA/use of hydroxyurea

A

Prevent sickling crises in SCA by causing production of HbF (alpha 2 gamma 2)

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141
Q

What is the common presenting sign of SCA in infants?

A

Dactylitis

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142
Q

What happens to the spleen over a long period of time in SCA?

A

Shrinks and becomes fibrotic

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143
Q

What is the most common cause of death in kids with SCA?

A

Infection with encapsulated organisms

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144
Q

What is the most common cause of death in adults with SCA?

A

Acute chest syndrome - vaso-occlusion in pulmonary microcirculation. Precipitated by pneumonia (hypoxia).

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145
Q

Gross hematuria and proteinuria in a patient with SCA

A

Renal papillary necrosis

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146
Q

What is the most common site of infarction in a patient with sickle cell trait and how does it present?

A

Renal medulla - microinfarctions lead to microscopic hematuria and decreased ability to concentrate urine.

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147
Q

What is the metabisulfite test?

A

Test for sickle cell anemia or trait- causes sickling of cells in either disorder.

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148
Q

What is hemoglobin C?

A

Autosomal recessive mutation in beta chain of hemoglobin - glutamic acid replaced by lysine (Hb C - lyCine).

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149
Q

What is seen on peripheral smear in a patient with hemoglobin C?

A

Peripheral HbC crystals due to glutamic acid being replaced by lysine (AR).

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150
Q

What are the two primary causes of extravascular hemolytic anemia?

A

SCA, HS

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151
Q

Ecalizumab - use and MOA

A

Treats PNH; mAB against complement C5

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152
Q

Why are RBCs not destroyed by complement?

A

Because they contain MIRL (membrane inhibitor of reactive lysis) and DAF (decay accelerating factor) on their surface. These are connected to the RBC by GPI, which is lost in PNH.

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153
Q

What is GPI, what function does it serve, and what is some pathology associated with it?

A

GPI anchors MIRL and DAF to the surface of the RBC to prevent it from being destroyed from complement. GPI is lost in PNH

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154
Q

What is the mechanism of inheritance of PNH?

A

Acquired (not inherited)

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155
Q

Triad of hemolytic anemia, pancytopenia and venous thrombosis

A

PNH

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156
Q

Why do patients with PNH get hemolysis at night?

A

Because shallow breathing during sleep causes a slight acidosis, which activates complement, allowing for destruction of RBCs that don’t have GPI anchoring MIRL and DAF to their surface.

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157
Q

What CD markers are lost in PNH?

A

CD55/CD59 (these use GPI as a linker molecule)

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158
Q

What is the main cause of death in PNH?

A

Thrombosis of hepatic, portal, or cerebral veins (platelets lack GPI).

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159
Q

What destroys cells in PNH?

A

Complement

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160
Q

Treatment for PNH and its MOA

A

Ecalizumab - C5 complement inhibitor

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161
Q

What is the function of G6PD in the RBC?

A

To produce NADPH for regeneration of reduced glutathione to prevent oxidative damage of RBCs.

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162
Q

How is G6PD deficiency inherited?

A

X-linked recessive

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163
Q

What is a Heinz body?

A

Precipitation of hemoglobin sulfhydryl groups in RBCs seen in G6PD deficiency.

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164
Q

What causes bite cells?

A

Removal of Heinz bodies by splenic macrophages in G6PD deficiency.

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165
Q

What are the two cell morphologies seen in G6PD deficiency?

A

Heinz bodies (precipitated hemoglobin) Bite cells (removal of Heinz bodies by splenic macrophages).

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166
Q

Where are RBCs primarily destroyed in G6PD deficiency?

A

Extravascularly

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167
Q

Warm agglutinin AIHA antibody

A

IgG

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168
Q

Cold agglutinin AIHA antibody

A

IgM

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169
Q

List 3 causes of warm agglutinin AIHA

A

SLE, CLL, methyldopa

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170
Q

What RBC morphology will be seen on warm agglutinin AIHA?

A

Spherocytes

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171
Q

List 2 infectious causes of cold agglutinin AIHA

A

Mycoplasma pneumoniae, mono

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172
Q

How do you do a direct Coombs test?

A

Add anti-Ig antibody to the patient’s serum. RBCs agglutinate if they are coated with Ig. (Do I have RBCs coated with Ig?)

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173
Q

How do you do an indirect Coombs test?

A

Add normal RBCs to a patient’s serum. Agglutination = serum has surface Ig. (Does the patient have antibodies in their serum?)

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174
Q

What cells are the hallmark of microangiopathic hemolytic anemia?

A

Schistocytes

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175
Q

What causes macroangiopathic hemolytic anemia and what cells are hallmark for it?

A

Typically, caused by RBCs shearing against cardiac valve prostheses and in aortic stenosis. Schistocytes

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176
Q

Hypocellular bone marrow on biopsy

A

Aplastic anemia

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177
Q

What cells are deficient in aplastic anemia?

A

RBCs, leukocytes, platelets

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178
Q

What cells are seen in a myelophthisic process?

A

Dakryocytes (teardrop cells)

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179
Q

Name the cells produced in the myeloid lineage

A

RBCs, neutrophils, basophils, eosinophils, monocytes, megakaryocytes

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180
Q

Name the cells produced in the lymphoid lineage

A

CD8/CD4 T cells, B cells, NK cells

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181
Q

What two drugs can be used to increase neutrophil count?

A

GM-CSF, G-CSF

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182
Q

Which cells in the blood are most sensitive to radiation?

A

Lymphocytes (check lymphocytes if you suspect radiation toxicity).

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183
Q

Decreased Fc receptor expression on neutrophils is suggestive of..

A

A left shift (decreased CD 16)

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184
Q

Immature neutrophils will have decreased numbers of what CD marker?

A

CD 16 - due to decreased Fc receptor expression

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185
Q

What causes eosinophilia seen in Hodgkin disease?

A

Increased IL-5 production

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186
Q

Basophilia is associated with…

A

CML

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187
Q

What bacteria causes a lymphocytic leukocytosis?

A

B. pertussis (produces a protein that prevents lymphocytes from leaving blood into the nodes)

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188
Q

What part of the lymph node is inflamed in mononucleosis?

A

Paracortex (where the CD8 T cells are)

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189
Q

What part of the spleen is inflamed/enlarged in mononucleosis?

A

Periarterial lymphatic sheath (PALS- where the T cells are)

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190
Q

What are heterophile antibodies?

A

IgM produced in mono that attacks RBCs from other species (sheep, horse). Tested in Monospot test.

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191
Q

What is screened for as a confirmatory test for EBV?

A

EBV viral capsid antigen

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192
Q

What defines an acute leukemia?

A

> 20% blasts in the bone marrow

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193
Q

What is the key marker to identify a lymphoblast?

A

TdT

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194
Q

What is TdT and what does it signify?

A

TdT is DNA polymerase. Its presence means that the cell is a lymphoblast.

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195
Q

What is an Auer rod composed of?

A

Crystallized myeloperoxidase - specific for a myeloblast, AML.

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196
Q

Crystallized myeloperoxidase seen in a cell

A

Auer rod - seen in myeloblasts of AML

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197
Q

Name the marker seen on each cell that would differentiate a myeloblast from a lymphoblast.

A

Myeloblast - myeloperoxidase (Auer rod) Lymphoblast - TdT (DNA polymerase)

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198
Q

What 2 leukemias are associated with Down syndrome?

A

Acute megakaryoblastic leukemia (before age of 5); ALL (after age of 5)

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199
Q

Name the 3 CD markers classically expressed by B cells in ALL

A

CD 10, CD 19, CD 20

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200
Q

CD 10, CD 19, CD 20 positive blasts

A

B cell ALL

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201
Q

What are two areas that must be closely watched or prophylaxed in patients with ALL?

A

Testes/CNS

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202
Q

What leukemia spreads to CNS?

A

ALL

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203
Q

t(12;21)

A

Good prognosis in ALL

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204
Q

Translocation associated with good prognosis in ALL

A

t(12;21)

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205
Q

Translocation associated with bad prognosis in ALL

A

t(9;22)

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206
Q

t(9;22) ALL

A

Bad prognosis

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207
Q

Name the CD markers seen in T cell ALL

A

CD2-CD8 (NOT CD10)

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208
Q

What are the “T’s” of T cell ALL?

A

Thymic mass in Teenager (actually a lymphoma because it is a mass)

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209
Q

In what leukemia do you prophylax the CNS and testes with chemo in kids?

A

ALL

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210
Q

t(15;17)

A

M3 AML (APL) - treat with ATRA

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211
Q

How does APL commonly present?

A

DIC

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212
Q

Smear shows cells with numerous Auer rods.. Dx?

A

M3 AML (APL) - treat with ATRA

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213
Q

What is the mutation seen in APL?

A

Disruption of the retinoic acid receptor, causing promyelocytes to accumulate.

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214
Q

What is the treatment for APL (M3 AML) and how does it work?

A

ATRA - works by binding retinoic acid receptor inducing differentiation in promyelocytes (causes them to mature)

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215
Q

What is the marker for a hematopoietic stem cell?

A

CD34

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216
Q

Acute monocytic leukemia typically infiltrates…

A

Gums (patients present with gum involvement)

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217
Q

Disorder associated with acute megakaryoblastic anemia.

A

Down syndrome (after the age of 5)

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218
Q

Abnormal myeloid maturation with increased blasts that comprise < 20% of cells

A

Myelodysplastic syndrome (can progress to AML if blasts > 20%).

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219
Q

What is the underlying pathophysiology of myelodysplastic syndrome?

A

Hypercellular bone marrow with cytopenias after radiation or chemotherapy

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220
Q

Abnormal myeloid maturation with increased blasts that comprise > 20% of cells

A

AML (<20% = myelodysplastic syndrome).

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4
5
Perfectly
221
Q

What is a pseudo-Pelger-Huet cell?

A

Bi-lobed PMN seen in myelodysplastic syndrome

222
Q

Chronic leukemia is a proliferation of…

A

Mature circulating lymphocytes

223
Q

Naive B cells expressing CD5 and CD 20

A

CLL

224
Q

Smudge cells

A

CLL

225
Q

CLL involving lymph nodes is known as…

A

Small lymphocytic lymphoma

226
Q

Why is infection the #1 cause of death in CLL?

A

Because neoplastic B cells don’t secrete Ig, resulting in hypogammaglobulinemia

227
Q

What is the Richter transformation?

A

CLL transforming into DLBCL (patient with CLL with rapidly enlarging spleen and LN).

228
Q

TRAP positive cells

A

Hairy cell leukemia

229
Q

Hairy cell leukemia cells are neoplastic proliferations of…

A

Mature B cells

230
Q

Treatment for hairy cell leukemia

A

Cladribine, also known as 2-CDA (guanosine)

231
Q

What causes splenomegaly in hairy cell leukemia?

A

Expansion of the red pulp

232
Q

What is the MOA and use of cladribine?

A

Adenosine deaminase inhibitor, causing adenosine to accumulate to toxic levels in neoplastic B cells. Used in hairy cell leukemia.

233
Q

Classic presentation of hairy cell leukemia

A

Dry tap, pancytopenia, splenomegaly, patient in 50s/60s

234
Q

Neoplasm associated with HTLV-1

A

ATLL (adult T cell leukemia/lymphoma)

235
Q

Cause of adult T cell leukemia/lymphoma and where it is seen geographically

A

HTLV-1 - Japan/Caribbean

236
Q

Patient presents with lytic bone lesions, hypercalcemia, and cutaneous rash. Dx?

A

Adult T cell lymphoma (don’t jump on multiple myeloma! HTLV-1 ATLL will cause a rash).

237
Q

Which malignant lymphoma shows involvement of the epidermis?

A

Mycosis fungoides

238
Q

What are Pautrier microabscesses?

A

Aggregates of neoplastic T cells in epidermis seen in MF/Sezary syndrome

239
Q

What is the immunophenotype of cells in mycosis fungoides?

A

CD3 and CD4 + (malignant neoplasm of T helper-inducer lymphocytes)

240
Q

What are Sezary cells?

A

Malignant T cells with cerebriform nuclei (nucleus looks like a brain).

241
Q

Malignant T cells with cerebriform nuclei

A

Sezary cells

242
Q

What is a myeloproliferative disorder?

A

Accumulation of mature myeloid cells (RBCs= PV, leukocytes = CML, platelets = essential thrombocytosis). Cells of ALL myeloid lineage will be increased, but the disorder is named for the predominant cell.

243
Q

t(9;22)

A

CML - Philadelphia chromosome (also bad prognosis in ALL)

244
Q

What is BRC-ABL?

A

Tyrosine kinase fused in CML

245
Q

<p>Treatment for CML</p>

A

<p>Imatinib - blocks intrinsic tyrosine kinase activity of BCR-ABL tyrosine kinase</p>

246
Q

Why do patients with CML progress to either AML or ALL?

A

Because the hematopoietic stem cell is what is actually mutated

247
Q

How is a leukemoid reaction differentiated from CML?

A

Leukemoid reaction = high leukocyte alkaline phosphatase, CML = low leukocyte alanine phosphatase; CML = increased basophils (LR not); CML = t(9;22) (LR not)

248
Q

What is a leukemoid reaction?

A

Increased WBC count with increased neutrophils, and HIGH leukocyte alkaline phosphatase (will be LOW in CML).

249
Q

What is the mutation in polycythemia vera?

A

JAK2 - tyrosine kinase

250
Q

What is Polycythemia?

A

Neoplastic proliferation of mature myeloid cells, especially RBCs

251
Q

Aquagenic pruritus

A

Polycythemia vera - itching after a shower (increased mast cells)

252
Q

What is a common cause of death in patients with polycythemia vera?

A

Budd-Chiari syndrome- thrombosis in hepatic vein resulting in liver infarction

253
Q

How is polycythemia vera differentiated from reactive polycythemia?

A

In reactive polycythemia, EPO would be high (due to hypoxia in lung disease or ectopic tumor production of EPO - renal cell CA). EPO will be low in PV.

254
Q

What is essential thrombocythemia?

A

Neoplastic proliferation of mature myeloid cells, especially platelets. Associated with JAK 2 kinase mutation

255
Q

What mutation causes essential thrombocythemia?

A

JAK 2 kinase

256
Q

What mutation causes myelofibrosis?

A

JAK 2 kinase

257
Q

Where are B cells present in the LN?

A

Cortex (outermost layer - expanded in bacterial infection)

258
Q

Where are T cells present in the LN?

A

Paracortex (expanded in viral infection)

259
Q

t(14;18)

A

Follicular lymphoma

260
Q

Translocation in follicular lymphoma

A

t(14;18)

261
Q

What is the molecular mechanism of the translocation in follicular lymphoma?

A

Translocation of the heavy chain Ig (14) and bcl-2 (18)

262
Q

Ig/bcl-2 translocation

A

Follicular lymphoma

263
Q

What is the normal function of bcl-2?

A

Stabilizes the mitochondrial membrane, preventing cytochrome C from leaking and causing apoptosis. Mutated in follicular lymphoma - lose apoptosis in B cells t(14;18).

264
Q

Why do LN lack tingible body macrophages in follicular lymphoma?

A

Apoptosis is lost because bcl-2 is lost. Would normally see macrophages consuming debris of B cells that were negatively selected in LN.

265
Q

t(11;14)

A

Mantle cell lymphoma

266
Q

Neoplastic proliferation of cells expanding the region immediately adjacent to the follicle in a LN.

A

Mantle cell lymphoma (mantle region is the region immediately adjacent to the follicle).

267
Q

What translocation is seen in mantle cell lymphoma?

A

t(11;14)

268
Q

What is overexpressed in mantle cell lymphoma?

A

Cyclin D

269
Q

Cyclin D overexpression

A

Mantle cell lymphoma

270
Q

What is the function of cyclin D?

A

Promotion of G1/S transition in cell cycle

271
Q

What proteins are fused due to the translocation in mantle cell lymphoma?

A

Cyclin D1 (11) and Ig heavy chain (14).

272
Q

What lymphoma is associated with chronic inflammatory states?

A

Marginal zone lymphoma (marginal zone is formed by activation of cells in chronic infection/inflammation).

273
Q

Sjogren syndrome is related to what lymphoma?

A

Marginal zone lymphoma

274
Q

Hashimoto thyroiditis is related to what lymphoma?

A

Marginal zone lymphoma

275
Q

H. pylori infection is related to what lymphoma?

A

MALToma (marginal zone lymphoma in mucosal sites)

276
Q

What are the two forms of Burkitt lymphoma and where do they typically arise?

A

African - Jaw, Sporadic - abdomen

277
Q

t(8;14)

A

Burkitt lymphoma

278
Q

Translocation in Burkitt lymphoma

A

t(8;14)

279
Q

What proteins are fused due to the translocation in Burkitt lymphoma?

A

c -myc oncogene (8) and Ig heavy chain (14)

280
Q

Starry sky appearance of lymphocytes

A

Burkitt lymphoma

281
Q

Infection that causes Burkitt lymphoma

A

EBV

282
Q

Histology of Burkitt lymphoma

A

Starry sky appearance

283
Q

What lymphoma can metastasize into the CNS?

A

DLBCL

284
Q

Describe the shape of growth of cells in DLBCL.

A

Sheets

285
Q

DLBCL can arise from what chronic leukemia?

A

CLL, via the Richter transformation

286
Q

What is the hallmark of Hodgkin’s lymphoma?

A

Reed-Sternberg cell

287
Q

Large B cell with a multilobed nucleus and prominent nucleoli

A

Reed-Sternberg cell

288
Q

What CD markers is a Reed-Sternberg cell positive for?

A

CD15 and CD30

289
Q

CD15 and CD30 positive B cell

A

Reed Sterngerg cell (HL)

290
Q

What causes B symptoms in HL?

A

Cytokine production by Reed-Sternberg cells

291
Q

What is the most common form of HL?

A

Nodular sclerosing (lacunar Reed-Sternberg cells)

292
Q

Lacunar cells

A

Reed Sternberg cell in nodular sclerosing HL (RS cell in a cleared out area - “lake).

293
Q

What form of HL has the best prognosis?

A

Lymphocyte rich

294
Q

What form of HL has the worst prognosis?

A

Lymphocyte depleted

295
Q

What is seen in mixed-cellularity HL and what causes it?

A

Abundant eosinophils, called in by IL-5

296
Q

RA + splenomegaly + neutropenia

A

Felty syndrome

297
Q

High serum IL-6 is associated with what blood neoplasm?

A

Multiple myeloma

298
Q

What type of lesions are seen on x-ray in a patient with multiple myeloma?

A

Punched out, lytic lesions

299
Q

How does multiple myeloma cause hypercalcemia?

A

Neoplastic plasma cells activate RANK receptor on osteoclasts

300
Q

What is the CRAB criteria?

A

hyperCalcemia; Renal insufficiency; Anemia; Bone lytic lesions/Bone pain - in multiple myeloma

301
Q

What causes increased serum protein in multiple myeloma?

A

Plasma cells secreting immunoglobulin

302
Q

What is seen on electrophoresis in multiple myeloma?

A

Spike in the gamma globulin (M-spike)

303
Q

What does an M spike mean?

A

Overproduction of a monoclonal immunoglobulin (NOT necessarily IgM).

304
Q

What is a Roleaux formation and what is it seen in?

A

Piled up RBCs seen in multiple myeloma

305
Q

Piled up RBCs seen in multiple myeloma

A

Roleaux formation

306
Q

<p>What is Bence Jones protein?</p>

A

<p>Free light chain (from Ig) excreted in urine.</p>

307
Q

What type of amyloidosis is seen in multiple myeloma?

A

Primary (AL)

308
Q

What substance is produced by the plasma cells in multiple myeloma to cause hypercalcemia?

A

Osteoclast-activating factor (also produces bone pain).

309
Q

What is MGUS?

A

Increased serum protein with M spike on SPEP but no other features of multiple myeloma.

310
Q

What is the rate of progression of MGUS to multiple myeloma?

A

1-2% per year

311
Q

Isolated M spike with no multiple myeloma symptoms.

A

MGUS (monoclonal gammopathy of undetermined significance)

312
Q

B cell lymphoma with monoclonal IgM production

A

Waldenstrom macroglobulinemia

313
Q

What is Waldenstrom macroglobulinemia?

A

B cell lymphoma with monoclonal IgM production.

314
Q

M spike due to IgM

A

Waldenstrom macroglobulinemia

315
Q

<p>Plasma cell dyscrasia causing hyperviscosity</p>

A

<p>Waldenstrom macroglobulinemia

| note that there's NO lytic bone lesions!</p>

316
Q

Birbeck granules on EM

A

Langerhans cell histiocytosis

317
Q

Tennis racket shaped granules on EM

A

Langerhans cell histiocytosis (Birbeck granules)

318
Q

What stain is used to detect Langerhans cell histiocytosis?

A

S100

319
Q

S100 positive cells

A

Langerhans cell histiocytosis (also melanoma, schwannoma). Also CD1a positive

320
Q

CD1a positive cells

A

Langerhans cell histiocytosis (also melanoma, schwannoma). Also S100 positive

321
Q

Presentation of Langerhans cell histiocytosis

A

In kids as lytic bone lesions and skin rash

322
Q

What type of receptor is JAK-2?

A

Non-receptor tyrosine kinase

323
Q

High Ki-67 fraction…

A

Burkit Lymphoma

324
Q

Name 3 NNRTIs

A

NeVIRapine, efaVIRenz, delaVIRidine (“vir” in the middle)

325
Q

What HIV drug causes nightmares?

A

EfaVIRenz (NNRTI)

326
Q

What is the dose-limiting side effect of AZT?

A

Bone marrow suppression

327
Q

Name 6 NRTI’s

A

Tenofovir; Emtricitabine; Abacavir; Lamivudine; Didanosine; Stavudine

328
Q

What suffix is indicative of a protease inhibitor?

A

ends in -navir (ritonavir, indinavir, etc.)

329
Q

What is ralTEGRAvir?

A

HIV inTEGRAse inhibitor

330
Q

Name 1 HIV integrase inhibitor

A

RalTEGRAvir

331
Q

What is emtracitabine?

A

NRTI

332
Q

What is abacavir?

A

NRTI (NOT a protease inhibitor because it ends in -avir, not -navir).

333
Q

What is zidovudine?

A

NRTI

334
Q

What is the dose-limiting side effect of abacavir?

A

Life threatening allergy (it is an NRTI)

335
Q

What is ritonavir?

A

Protease inhibitor (-navir)

336
Q

What is indinavir?

A

Protease inhibitor (-navir)

337
Q

What is saquinavir?

A

Protease inhibitor (-navir)

338
Q

What is the main side effect of protease inhibitors?

A

Disordered lipid and CHO metabolism with central adiposity (buffalo hump) and insulin resistance.

339
Q

Name a fusion inhibitor.

A

EnFUrvatide (FUsion inhibitor)

340
Q

What is enFUrvatide?

A

Fusion inhibitor

341
Q

Name a drug that binds gp41

A

EnFUrvatide (FUsion inhibitor)

342
Q

What is maraviroc?

A

CCR5 inhibitor

343
Q

Name a CCR5 inhibitor

A

Maraviroc

344
Q

What is a unique side effect of indinavir?

A

Nephrolithiasis

345
Q

What is neVIRapine?

A

NNRTI (vir in the middle)

346
Q

What is efaVIRenz?

A

NNRTI (vir in the middle)

347
Q

What is delaVIRidine?

A

NNRTI (vir in the middle)

348
Q

How do NNRTIs work?

A

They bind to reverse transcriptase at a site different from NRTIs. They don’t require phosphorylation.

349
Q

What antiretroviral is contraindicated in pregnancy?

A

efaVIRenz (NNRTI) - risk of NTD’s.

350
Q

<p>What should be given to babies born to HIV positive mothers?</p>

A

<p>AZT (zidovudine)</p>

351
Q

What is erythrocytosis?

A

polycythemia = increased hematocrit

352
Q

what is the life span of a platelet?

A

8-10 days

353
Q

What is the vWF receptor and what is the fibrinogen receptor?

A

vWF binds GpIb

Fibrinogen binds GpIIb/IIIa

354
Q

what is the normal leukocyte count?

A

4000 - 10,000 cells/mm3

355
Q

What is the WBC differential from highest to lowest?

A 
Neutrophils Like Making Everything Better
Neutrophils (54-62%)
Lymphocytes (25-33%)
Monocytes (3-7%)
Eosinophils (1-3%)
Basophils (0.0.75%)
356
Q

What are band cells?

A

Increased band cells (immature neutrophils) reflect states of increased myeloid proliferation like in bacterial infections or CML

357
Q

What are contained in the small more numerous granules of neutrophils vs the larger less numerous azurophilic granules (lysosomes)?

A

Small more numerous specific granules: alkaline phosphatase, collagenase, lysozyme, and lactoferrin

Larger less numerous azurophilic granules (lysosomes): acid phosphatase, peroxidase, and beta glucuronidase

358
Q

which cell type contains a large kidney shaped nucleus?

A

Monocyte

359
Q

what are the cell surface markers found on macrophages?

A

CD14, B7, MHC1, MHC2, CD40

360
Q

which cytokine activates macrophages?

A

interferon gamma

361
Q

What is CD14?

A

Cell surface marker for macrophages - CD14 binds to LPS on gram negative bacteria and induces macrophages to make acute phase cytokines (IL1, IL6 and TNF alpha)

362
Q

what do eosinophils use to defend against helminthic infections?

A

major basic protein

363
Q

Which cell type is “highly phagocytic for antigen-antibody complexes”?

A

Eosinophils

364
Q

What are histaminase and arylsulfatase?

A

Produced by eosinophils to help limit the reaction following a mast cell degranulation

365
Q

List 5 cases of eosinophilia

A 
NAACP
Neoplastic
Asthma
Allergic processes
Collagen vascular diseases
Parasites (invasive)
366
Q

What is contained in granules within basophils?

A

heparin, histamine and leukotrines (LTD4)

367
Q

Which cell type mediates an allergic reaction ?

A

Basophils or mast cells in local tissues

368
Q

What enzyme can you follow as an activator of mast cell activation?

A

tryptase

369
Q

Which cell type functions as a link between the innate and adaptive immune system?

A 
Dendritic cells
Express MHC class II and Fc receptor on surface

note that these are called Langerhans cells in the skin

370
Q

Can B lymphocytes function as an APC?

A

yes they contain MHC2

371
Q

which cell type contains an off center nucleus with a clock face chromatin distribution?

A

Plasma cells

372
Q

Which cell type mediates the cellular immune response?

A

T cells

373
Q

Which cytokine is necessary for T cell activation?

A

CD28 (costimulatory signal)

374
Q

What type of antibodies are anti-A and anti-B?

A

IgM antibodies - do not cross the placenta

375
Q

What type of antibody is anti-Rh?

A

IgG antibody - can cross the placenta

376
Q

Which blood type is the universal donor? Universal acceptor?

A

Universal donor of RBCs: Type O
Universal donor of plasma: AB
Universal recipient of RBCs: AB
Universal recipient of plasma: O

377
Q

Describe Blood type A

A

Blood type A contains the A antigen on the RBC surface and anti-B antibodies in the plasma

378
Q

Describe blood type AB

A

Has both A and B antigens on the RBC surface and no antibodies in the plasma

as compared to blood type O which has neither A nor B antigens on the RBC surface but has both antibodies in the plasma

379
Q

What is Rhogam?

A

IgG anti Rh antibodies given at 28 weeks gestation and immediately post-partum to block maternal immune response to the foreign fetal RhD antigens - it prevents the maternal antibodies from recognizing the fetal RBCs

You want to prevent hemolytic disease in the NEXT Rh+ child

380
Q

What is the hypersensitivity if you transfuse the wrong blood type?

A

Type II hypersensitivity

381
Q

How does Rhogam work?

A

Binds to the fetal RBCs that have gotten intot he mothers body and prevents exposure of the surface Rh-D antigen to the moms immune system

382
Q

List the coagulation factors in the extrinsic pathway

A

7

383
Q

List the coagulation factors in the intrinsic pathway

A

12, 11, 9, 8

384
Q

What does PT and PTT measure?

A

PT measures the extrinsic pathway

PTT measures the intrinsic pathway

385
Q

What are accelerating factors?

A

Factor VIIIa and Factor Va - both are inhibited by protein C and protein S

386
Q

What carries and protects Factor VIII?

A

vWF

387
Q

What is epoxide reductase?

A

Enzyme that catalyzes oxidized vitamin K to reduced vitamin K so it can activated 2, 7, 9, 10, C and S

388
Q

What does antithrombin do?

A

antithrombin inhibits activated forms of factors 2, 7, 9, 10, 11 and 12

antithrombin is activated by heparin

389
Q

How is it possible for Factor V to be resistant to inhibition by protein C?

A

Factor V Leiden mutation

390
Q

name 4 anti-aggregation factors that stop platelet aggregation

A

PGI2 and NO - released by endothelial cells
Increased blood flow
decreased platelet aggregation

391
Q

What do ticlodipine and clopidogrel inhibit?

A

ADP induced expression of GpIIb/IIIa expression

392
Q

What does Aciximab inhibit?

A

Directly inhibits GpIIb/IIIa

393
Q

what is clopidogrel synergistic with?

A

Warfarin

clopidogrel decreases the metabolism of warfarin making it stick around longer

394
Q

Do you have an increased or decreases ESR in polycythemia vera?

A

decreased

also decreased in sickle cell anemia, congestive heart failure, microcytosis and hypofibrinogenemia

395
Q

What is the effect of TXA2?

A

Vasoconstriction and platelet aggregation

396
Q

What is an acanthocyte and when will you see one?

A

Spur cell, RBC with irregular spikes

seen in liver disease, abetalipoproteinemia

397
Q

When do you see basophilic stippling in a RBC?

A

Thalassemias, Anemia of chronic disease and lead poisoning
also alcohol abuse

Caused by inhibition of the enzyme that breaks down RNA - so you get clumps of denatured RNA in the RBC

398
Q

What is a Bite cell and when do you see it?

A

Seen in G6PD deficiency

Due to removed Heinz bodies from cells by the spleen

399
Q

What is an elliptocyte?

A

pencil shaped RBC - hereditary elliptocytosis

400
Q

What is a ringed sideroblast?

A

RBC with excess iron IN THE MITROCHONDRIA
due to sideroblastic anemia (lead poisoning, drugs, genetic conditions, myelodysplastic syndromes)

due to a protoporphyrin deficiency

401
Q

what is a schistocyte?

A

Helmet cells

seen in DIC, TTP/HUS, and traumatic hemolysis

402
Q

What causes a tear drop cell?

A

Bone marrow infiltration (like myelofibrosis)

403
Q

List 4 causes of target cells

A 
"THAL"
Thalassemia
HbC disease
Asplenia
Liver disease
404
Q

What are Heinz bodies?

A

Oxidation of iron from ferrous to ferric form leads to denatured hemoglobin precipitation and damage to RBC membrane
Leads to formation of bite cells

405
Q

What is a Howell Jolly body?

A

Basophilic nuclear remnants found in RBCs

Seen in patients who don’t have a spleen (hyposplenia, asplenia) or after mothball ingestion (naphthalene)

406
Q

List the 5 causes of microcytic anemia?

A

Iron deficiency, anemia or chronic disease (may start as normocytic), thalassemias, lead poisoning, sideroblastic anemia

407
Q

List the 3 causes of non-hemolytic normocytic anemia

A

Anemia of chronic disease (only starts as normocytic), Aplastic anemia and Chronic kidney disease

408
Q

List the 5 causes of intrinsic hemolytic normocytic anemia?

A

RBC membrane defect (hereditary spherocytosis), RBC enzyme deficiency (G6PD, PK), HbC, Sickle cell anemia, Paroxysmal nocturnal hemoglobinuria

409
Q

List the 4 causes of extrinsic hemolytic normocytic anemia

A

Autoimmune, microangiopathic, macroangiopathic, infections

410
Q

List the 6 causes of macrocytic anemia?

A

Megaloblastic - Folate deficiency, B12 deficiency, orotic aciduria
Non-megaloblastic - Liver disease, alcoholism, Reticulocytosis

411
Q

What are the findings in a patient with iron deficiency anemia?

A

decreased iron, increased TIBC and decreased ferritin

412
Q

What is the trans deletion seen in alpha thalassemia?

A

prevalent in African populations.

leads to one deletion on each chromosome

413
Q

Which population commonly gets Beta thalassemia mutations?

A

Point mutations in splice sites and promoter sequences that lead to decreased Beta globin synthesis

Prevalent in Mediterranean populations

414
Q

Which beta thalassemia results in absent beta chain?

A

beta thalassemia major leads to increased HbF

patient will require transfusions - leads to secondary hemochromatosis

415
Q

What is the defect that occurs in lead poisoning?

A

Lead inhibits ferrochelatase and ALA dehydratase leading to decreased heme synthesis

  • also inhibits rRNA degradation causing RBCs to retain aggregates of rRNA (basophilic stippling)
416
Q

What are the clinical findings in a patient with lead poisoning?

A

LEAD
Lead Lines on gingiva (burton’s lines) and on metaphyses of long bones on Xray
Encephalopathy and Erythrocyte basophilic stippling
Abdominal colic and sideroblastic Anemia
Drops - wrist drop and foot drop.

417
Q

What is the treatment for lead poisoning?

A

Dimercaprol and EDTA are first line of treatment

SUCcimer is used for chelation for kids (it SUCks to be a kid who eats lead)

418
Q

what are the lab findings in sideroblastic anemia?

A

increased iron, normal TIBC and increased ferritin

419
Q

What is the treatment for sideroblastic anemia?

A

Pyridoxine (B6) which is cofactor for d-ALA synthase

420
Q

What are the hereditary and reversible causes of sideroblastic anemia?

A

Hereditary - X linked defect in d-ALA synthase gene

Reversible - alcohol, lead, and isoniazid

421
Q

List 3 drugs that can cause folate deficiency

A

Methotrexate, trimethoprim, and phenytoin

422
Q

What fluke can lead to B12 deficiency?

A

Diphyllobothrium latum - fish tapeworm

note that proton pump inhibitors, crohn’s disease and vegans can also get this, pernicious anemia

423
Q

Why do patients with B12 deficiency get neurologic symptoms?

A

Subacute combined degeneration due to involvement of B12 in fatty acid pathways and myelin synthesis

leads to peripheral neuropathy with sensorimotor dysfunction, posterior columns (loss of vibration/proprioception), lateral corticospinal tracts (spasticity) and dementia

424
Q

children have megaloblastic anemia that hasn’t been cured by folate or B12 - what’s wrong with them?

A

Orotic aciduria - deficiency of UMP synthase which is involved in pyrimidine synthesis.
TX with uridine monophosphate to bypass mutated enzyme

note that these patients have hypersegmented neutrophils, glossitis, and orotic acid in urine

425
Q

Describe what happens in anemia of chronic disease

A

Inflammation increases IL6 leading to the liver making hepcidin.
Increases hepcidin inhibits ferroportin on intestinal mucosal cells and macrophages therefore inhibiting iron transport leading to decreased release of iron from macrophages

426
Q

what can cause aplastic anemia?

A
  • radiation and drugs (benzene, chloramphenicol, alkylating agents, antimetabolites)
  • viral agents (parvovirus B19, EBV, HIV, HCV)
  • Fanconi’s anemia (DNA repair defect)
  • idiopathic (immune mediated, primary stem cell defect) may follow acute hepatitis
427
Q

why does chronic kidney disease lead to nonhemolytic normocytic anemia?

A

Due to decreased erythropoietin leading to decreased hematopoiesis

428
Q

what is the inheritance pattern of hereditary spherocytosis?

A

AD

429
Q

What are the findings in hereditary spherocytosis?

A

Increased MCHC, increased red cell distribution width, positive osmotic fragility test. normal to decreased MCV with abundance of cells - masks microcytia

430
Q

What is the inheritance pattern of G6PD deficiency?

A

X linked

431
Q

A patient has back pain and then a few days later develops hemoglobinuria. what would you see on blood smear?

A

This is G6PD deficiency

you’ll see Heinz bodies and bite cells

432
Q

which disease has polymerized hemoglobin and deformed cell membrane?

A

Sickle cell disease

433
Q

What is pyruvate kinase deficiency?

A

AR deficiency leads to decreased ATP and rigid RBCs - can cause hemolytic anemia in a newborn

434
Q

What is the triad seen in paroxysmal nocturnal hemoglobinuria?

A

Hemolytic anemia, pancytopenia and venous thrombosis

Positive Ham’s test - red cell lysis at decreased pH due to activation of complement
Labs will show CD55(DAF)/59 - RBCs

435
Q

What is the treatment for a patient with paroxysmal nocturnal hemoglobinuria?

A

Eculizumab - inhibits complement

436
Q

What is the inheritance pattern of sickle cell disease?

A

Autosomal recessive, mutation is on chromosome 11

437
Q

what is the treatment for sickle cell anemia?

A

Hydroxyurea (increases HbF) and bone marrow transplant

438
Q

What would the treatment of salmonella osteomyelitis in a sickle cell patient be?

A

Cirpfloxacin or another fluoroquinolone

also do coverage against Staph aureus (naficillin/oxacillin, cefazolin, vancomycin)

439
Q

What is the warm and cold agglutinins seen in autoimmune hemolytic anemia?

A

Warm agglutinin - IgG

Cold agglutinin - IgM

440
Q

When do you see warm agglutinin Autoimmune hemolytic anemia?

A

chronic anemia seen in SLE, CLL or with certain drugs like alpha methyldopa

441
Q

When do you see cold agglutinin autoimmune hemolytic anemia?

A

Acute anemia triggered by the cold, seen in CLL, mycoplasma pneumonia infections or infectious mononucleosis (EBV)

442
Q

What are the lab findings in a patient with hemochromatosis?

A

Increases serum iron (if primary), decreased TIBC, increased ferritin, increased % saturation

443
Q

What are the lab findings in a patient with pregnancy or on OCPs?

A

increased TIBS, decreased transferrin saturation

444
Q

What are the lab findings in a patient with sideroblastic anemia?

A

increased serum iron, decreased TIBC, normal or increased ferritin, increased %saturation

445
Q

What is the affected enzyme in a patient with acute intermittent porphyria?

A

Porphobilinogen deaminase

446
Q

What are the symptoms in a patient with acute intermittent porphyria?

A 
5 Ps
painful abdomen
port wine colored urine
polyneuropathy
psychological disturbances
Precipitated by drugs (barbiturates, seizure drugs, rifampin, metoclopramide)
447
Q

What is the treatment in a patient with acute intermittent porphyria?

A

glucose and heme which inhibit ALA synthase

448
Q

What is porphyria cutanea tarda?

A

Defected uroporphyrinogen decarboxylase leading to accumulation of uroporphyrin (tea colored urine)

patients have blistering cutaneous photosensitivity. This is the most common porphyria

449
Q

What are the lab changes in hemophilia A and B

A

Increased PTT

450
Q

What are the clinical findings in patients with hemophilia A or B?

A

Macrohemorrhage in hemophilia - hemarthroses (bleeding into joints), easy brusing, increased PTT

451
Q

What are the lab changes in a patient with Vitamin K deficiency?

A

Increased PT and PTT

452
Q

What is seen with platelet abnormalities?

A

microhemorrhage: mucous membrane bleeding, epistaxis, petechiae, purpura, increased bleeding time and POSSIBEL decreased in platelet count

453
Q

What are the lab findings in a patient with Bernard-Soulier syndrome?

A

Decreased platelet count and increased bleeding time

454
Q

What are the lab findings in a patient with Glanzmann’s thrombasthenia?

A

Increased bleeding time. Blood smear will show no platelet clumping. note that platelet count is NORMAL

455
Q

What are the labs seen in idiopathic thrombocytopenic purpura?

A

decreased platelet count and increased bleeding time. Note that there will be increased megakaryocytes

456
Q

What are the lab findings in thrombotic thrombocytopenic purpura?

A

decreased platelet count, increased bleeding time and increased LDH. the patient will also have schiztocytes

457
Q

What is the treatment for Hemophilia A? for vWF disease?

A

Desmopressin - promotes release of factor VIII and vWF and t-PA from endothelial cells

458
Q

What are the symptoms in Thrombotic thrombocytopenic purpura?

A

FAT RN

Fever, microangiopathic hemolytic Anemia, Thrombocytopenia, Renal symptoms, Neurological symptoms

459
Q

What are the lab findings in a patient with vWF?

A

increased bleeding time and normal or increased PTT

460
Q

What are the lab findings in a patient with DIC?

A

decreased platelet count, increased bleeding time, increased PT and increased PTT

The patient will also have schistocytes, increased fibrin split products (D-dimers), decreased fibrinogen, decreased factors V and VIII

461
Q

Name 4 diseases that are hereditary thrombosis syndromes leading to hypercoagulability

A

Factor V leiden, Prothrombin gene mutation, Antithrombin deficiency, and Protein C or S deficiency

462
Q

What is factor V leiden disease?

A

Production of mutant factor V that is resistant to degradation by activated protein C. Most common cause of inherited hypercoagulability in whites

So factor V stays activated longer leading to increased coagulation

463
Q

What is Prothrombin gene mutation?

A

Mutation in 3’ untranslated region leading to increased production of prothrombin and therefore increased plasma levels and venous clots
G20210A

464
Q

What is antithrombin deficiency?

A

Inherited deficiency of antithrombin that has no direct effect on PT, PTT, or thrombin time but diminishes the increase in PTT following heparin administration - these patients are probably resistant to heparin in these patients

465
Q

What is Protein C or S deficiency?

A

decreased ability to inactivate factors V and VIII. Increased risk of thrombotic skin necrosis with hemorrhage following administration of warfarin

466
Q

Whats the point of fresh frozen plasma?

A

Increases coagulation factor levels - used for DIC< warfarin overdose or cirrhosis

467
Q

What is the point of giving cryoprecipitate?

A

Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin. used to treat coagulation factor deficiencies involving fibrinogen and factor VIII

468
Q

What is the lymphocyte predominant type of Hodgkin’s lymphoma?

A

Least common but has the best prognosis - found in younger men

469
Q

What is the nodular sclerosing type of Hodgkin’s lymphoma?

A

Most common type, good prognosis, biopsy shows bands of collagen, sclerosis and fibrosis, seen in young adults more common in females

470
Q

What is the mixed cellularity type of Hodgkin’s lymphoma?

A

Second most common, has the worst prognosis

471
Q

What is the lymphocyte depleted type of Hodgkin’s lymphoma?

A

very poor prognosis

472
Q

What is the translocation in burkitt’s lymphoma?

A

c-myc and heavy chain t(8;14)

8urk14

473
Q

What is the most common adult non Hodgkin lymphoma?

A

Diffuse large B cell lymphoma

474
Q

what type of lymphoma is caused by HTLV-1?

A

Adult T-cell lymphoma

this is aggressive- adults present with cutaneous lesions - especially effects populations in japan, west Africa and the caribbean

475
Q

What type of amyloidosis appears in multiple myeloma?

A

Primary amyloidosis (AL)

476
Q

What is seen on a blood smear of multiple myeloma?

A

Clock face chromatin and whitish intracytoplasmic inclusions containing immunoglobulins

477
Q

What is the treatment for hairy cell leukemia?

A

Cladribine - an adenosine analog

478
Q

What are auer rods?

A

Peroxidase positive cytoplasmic inclusions in granulocytes and myeloblasts. Commonly seen in acute promyelocytic leukemia (M3). Treatment of AML M3 can release Auer rods leading to DIC

479
Q

A child presents with lytic bone lesions and a skin rash - what might it be?

A

Langerhands cell histiocytosis

480
Q

What do you follow when administering heparin?

A

PTT

Heparin is a cofactor for the activation of antithrombin - leads to decreased thrombin and factor Xa

481
Q

What do you follow for warfarin?

A

PT

482
Q

why can you use heparin during pregnancy?

A

It doesn’t cross the placenta

483
Q

what is the antidote to heparin overdose?

A

Protamine sulfate (positively charged molecule that binds negatively charged heparin)

484
Q

How does low molecular weight heparin work?

A

Acts more on factor Xa - has better bioavailability and 2-4 times longer hald life than heparin. Can be administered subcutaneously without lab monitoring

485
Q

What is Heparin induced thrombocytopenia?

A

Development of IgG antibodies against heparin bound to platelet factor 4. The antibody-heparin-PF4 complex activates platelets leading to thrombosis and thrombocytopenia

seen in a patient that’s on heparin and their platelet counts are dropping

486
Q

What is the MOA of warfarin?

A

interferes with normal synthesis and gamma-carboxylation of vitamin K dependent clotting factors 2, 7, 9, 10 C and S

487
Q

What do you give for warfarin overdose?

A

Vitamin K

488
Q

What is a possible effect of warfarin?

A

Skin necrosis

489
Q

What is the MOA of thrombolytics

A

Directly or indirectly aid conversion of plasminogen to plasmin which cleaves thromobin and fibrin clots

Increases PT and PTT but does not change the platelet count

490
Q

how do you treat overdose of thrombolytics?

A

Aminocarpoic acid

491
Q

How does aspirin work?

A

Irreversible inhibits cyclooxygenase enzyme by covalent acetylation.
Increases bleeding time, decreases TXA2 and prostaglandins.
NO effect on PT or PTT

492
Q

what is the clinical use of aspirin?

A

antipyretic, analgesic, anti-inflammatory, antiplatelet

493
Q

name 4 ADP receptor inhibitors

A

Clopidogrel, ticlopidine, prasugral, ticagrelor

494
Q

How do ADP receptor inhibitors work?

A

Inhibit platelet aggregation by irreversible blocking ADP receptors.
Inhibit fibrinogen binding by preventing glycoprotein IIb/IIIa from binding to fibrinogen

495
Q

What do Cilostazol and dipyridamole do?

A

Phosphodiesterase III inhibitors, increased cAMP in platelets thus inhibiting platelet aggregation, they also cause vasodilation

used for patients with intermittent claudication

496
Q

name 3 GPIIb/IIIa inhibitors?

A

Abciximab, eptifibatide, tirofiban

497
Q

which antineoplastic drug acts by decreasing thymidine synthesis?

A

Methotrexate, 5-FU

498
Q

Which antineoplastic drug acts by decreasing purine synthesis?

A

6-MP

499
Q

Which antineoplastic drug acts by inhibiting topoisomerase II??

A

Etoposide

500
Q

Which antineoplastic drug inhibits microtubule formation?

A

Vinca alkaloids

501
Q

Which antineoplastic drug inhibits microtubule disassembly?

A

Paclitaxel

502
Q

how does methotrexate work?

A

Folic acid analog that inhibits dihydrofolate reductase leading to decreased dTMP therefore decreased DNA and protein synthesis

503
Q

how does 5-FU work?

A

Pyrimidine analog bioactivated to 5F-dUMP which covalently complexes folic acid. This complex inhibits thymidylate synthase leading to decreased dTMP and therefore decreased DNA and protein synthesis

504
Q

How do you treat an overdose with 5-FU?

A

rescue with thymidine

505
Q

What is Cytarabine?

A

arabinofuranosul cytidine

pyrimidine analog that leads to inhibition of DNA polymerase

506
Q

What is the MOA of doxorubicin and daunorubicin?

A

Generates free radicals, noncovalently intercalates in DNA leading to breaks in DNA and decreased replication

507
Q

What can you use to prevent cardiotoxicity when taking doxorubicin?

A

Dexrazoxane (iron chelating agent) used to prevent cardiotoxicity

508
Q

What is Dactinomycin?

A

Actinomycin D - intercalactes in DNA
used for Childhood tumors like Wilm’s tumor, Ewing’s sarcoma, rhabdomyocarcoma

children ACT out = dACTinomycin

509
Q

What is the MOA of bleomycin?

A

Induces free radical formation which causes breaks in DNA strands

510
Q

What is the clinical use of bleomycin?

A

Testicular cancer and Hodgkin’s lymphoma

511
Q

What are the side effects of using Bleomycin?

A

Pulmonary fibrosis, skin changes

512
Q

What is the MOA of cyclophosphamide and ifosfamide?

A

Covalently X link DNA at guanine N-7

Requires bioactivation by the liver!

513
Q

What are the side effects of cyclophosphamide and ifosfamide?

A

Myelosuppression, hemorrhagic cystitis (due to toxic metabolite acrotein)

514
Q

What is the toxic metabolite of cyclophosphamide and ifosfamide and how do you prevent it?

A

Toxic metabolite if acrotein - partially prevented with mesna - the thiol group in mesna binds to the toxic metabolite

515
Q

What are the Nitrosureas?

A

Carmustine, lomustine, semustine and streptozocin

mustard gas

516
Q

what is the MOA of nitrosureas and the side effects?

A

Requires bioactivation and crosses the blood brain barrier into the CNS so it is used to TX brain tumors like glioblastoma multiforme
but because it crosses the BBB it has CNS toxicity like ataxia and dizziness

517
Q

WHat is the MOA of Busulfan?

A

Alkylates DNA

518
Q

What is the used for Busulfan?

A

CML. Also used to ablate patient’s bone marrow before a bone marrow transplant

519
Q

What are the side effects of Busulfan?

A

Pulmonary fibrosis and hyperpigmentation

520
Q

what are Paclitaxel and Doetaxel used to treat?

A

Ovarian and breast carcinomas

521
Q

what is the MOA of Vincristine and Vinblastine?

A

Alkaloids that bind to tubulin in the M phase and block polymerization of microtubules \so that mitotic spindles can’t form (no cell division)

522
Q

What is the side effects of Vincristine and Vinblastine?

A

Vincristine - neurotoxicity leading to areflexia, peripheral neuritis. and also paralytic ileus

Vinblastine - Blasts bone - (bone marrow suppression)

523
Q

What is the MOA of Paclitaxel and Docetaxel?

A

Hyperstabilizes polymerized microtubules in the M phase so that the mitotic spindle can’t break down - anaphase can’t occur

524
Q

What is the MOA and use of Cisplatin, carboplatin and oxaliplatin?

A

Cross link DNA

used for testicular, bladder, ovary and lung carcinomas

525
Q

what is the MOA of etoposide and teniposide?

A

Inhibits topoisomerase II leading to increased DNA degradation

remember that Topoisomerase II is used to unwind supercoils as you unwind DNA

526
Q

WHat is the toxicity of Cisplatin and carboplatin?

A

Nephrotoxicity and acoustic nerve damage

527
Q

How can you prevent nephrotoxicity when using cisplatin and carboplatin?

A

Amifostine (free radical scavenger) and chloride diuresis

528
Q

What are the nephrotoxic/ototoxic drugs?

A

Vancomycin
Aminoglycosides
Loop diuretics
Cisplastin/carbplastin/oxaliplatin

529
Q

How do you treat testicular cancer?

A

Eradicate Ball Cancer

Etoposide, Bleomycin (or ifosfamide) and Cisplastin

530
Q

WHat are the side effects of etoposide and teniposide?

A

Myelosuprression, GI irritation and ALOPECIA

531
Q

What is the MOA of hydroxyurea?

A

Inhibits ribonucleotide reductase leading to decreased DNA synthesis (S-phase specific)

532
Q

What are the uses for Hydroxyurea?

A

Melanoma, CML and sickle cell disease (increases HbF)

533
Q

What are the side effects of using hydroxyurea?

A

bone marrow suppression and GI upset

534
Q

What is the MOA of prenisone and prednisolone?

A

May trigger apoptosis. may even work on nondividing cells
This is the most commonly used glucocorticoid in cancer chemotherapy
used in CLL and non-Hodgkin’s lymphomas

535
Q

Name two SERMs

A

Tamoxifen and raloxifene

536
Q

What’s the MOA of tamoxifen?

A

ER antagonist in the breast
ER agonist in the bone
ER partial agonist in the endometrium

537
Q

What is the MOA of Raloxifene?

A

ER agonist in the bone (TX osteoporosis)

ER antagonist in the breast and endometrium

538
Q

What are the differences in the toxicity between Tamoxifen and Raloxifene?

A

Tamoxifen - partial agonist in the endometrium so it can increased the risk of endometrial cancer, can cause hot flashes

Raloxifene - no increase in endometrial carcinoma because it’s an endometrial antagonist

539
Q

What is the MOA of Trastuzumab?

A

AKA Herceptin
Monoclonal antibody against HER-2 (c-erbB2) a tyrosine kinase,
Helps kill breast cancer cells that overexpress HER-2 possibly through antibody dependent cytoxicity

540
Q

What is the toxicity of Trastuzumab?

A

Cardiotoxicity

Herceptin hurts the heart

541
Q

What is the MOA of imatinib?

A

Philadelphia chromosome bcr-abl tyrosine kinase inhibitor

542
Q

What is the side effect of imatinib?

A

fluid retention

543
Q

WHat is the MOA of Rituximab?

A

Monoclonal antibody against CD20 which is found on most B cell neoplasms

544
Q

What is the clinical used of Rituximab?

A

Non-Hodgkin’s lymphoma, Rheumatoid arthritis (with methotrexate) and Pemphigus vulgaris, vasculitis, ITP

545
Q

WHat is the MOA of Vemurafenib?

A

Small molecule inhibitor of forms of the B-Raf kinase with the V600E mutations

546
Q

What is the clinical use of Vemurafenib?

A

Metastatic melanome

547
Q

What is the MOA of Bevacizumab?

A

Monoclonal antibody against VEGF so it inhibits angiogenesis

used for solid tumors

548
Q

Name two neoplastic drugs that can cause cardiotoxicity?

A

Doxorubicin and Trastuzumab

549
Q

Which drug can cause hemorrhagic cystitis?

A

Cyclophospamide

550
Q

List three drugs that can cause myslosuppression?

A

5-FU
6-MP
Methotrexate

551
Q

List two drugs that are both nephrotoxic and can also cause acoustic nerve damage

A

Cisplastin, Carboplatin

First Aid Chs Part 2 (6 decks)

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