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Step 1 AB > Heme/Lymph > Flashcards

Heme/Lymph Flashcards

1
Q

What triggers coagulation in extrinsic pathway?

A

Tissue injury releases tissue factor (thromboplastin)

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2
Q

What is deficiency of Hemophilia B?

A

Coagulation factor IX (9) deficiency

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3
Q

What is deficiency of Hemophilia A?

A

Factor 8

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4
Q

What is rate limited of coagulation cascade?

A

Convergence of extrinsic and intrinsic at Factor 10

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5
Q

Function of Protein C and S?

A

Inhibit Factors Va and VIIIa

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6
Q

Why does Warfarin transiently increase clot formation?

A

Warfarin inhibits Factors 2, 7, 9 and 10; but it also inhibits protein C and S MORE rapidly than clotting factors –> this transiently increases clot formation before the other factors are inhibited
-This is why we start Heparin first

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7
Q

What is the final common pathway?

A

Factor 10 –>10a
10a + 5a activate prothrombin to thrombin
Thrombin + 13a activate fibrin monomers into fibrin mesh

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8
Q

What is function of Antithrombin?

A

Inhibits thrombin from activating fibrin

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9
Q

What drug increases effectiveness of thrombin?

A

Heparin

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10
Q

How does coagulase affect coagulation pathway? What produces coagulase?

A

Coagulase activates thrombin

Made by Staph aureus

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11
Q

What other ions/proteins are necessary for coagulation cascade?

A

Calcium

Phospholipid

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12
Q

What are the functions of factor 12a?

A

1) 12a activates 11 –> 11a
2) 12a converts prekallikrein to kallikrein –> then kallikrein cleaves plasminogen to form plasmin –> plasmin breaks down fibrin mesh
- Kallikrein also converts HMWK to bradykinin –> Bradykinin vasodilates, increases vascular permeability and mediates pain (coagulation and inflammation are interconnected)

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13
Q

What does the prothrombin time measure?

A

Take plasma and add tissue factor –> measure how long it takes clot to form
Tests how effectively tissue factor can activate the tissue factor pathway and the final common pathway
Testing factors 7, 10, 5 and 2 (prothrombin)
Always reported as INR (1 is normal)

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14
Q

What does partial thromboplastin time measure?

A

Plasma + silica or something that activates tissue activating pathway

  • Also have to add phospholipids that normally combine with tissue factor to form thromboplastin
  • Tests function of contact activation pathway and final common pathway
  • Tests 12, 11, 9 and 8, 10, 5 and 2
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15
Q

What does bleeding time measure?

A

Should take 2-9 minutes
A function of platelet function
Disorders of coagulation cascade will not affect bleeding time

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16
Q

Hemophilia A and Hemophilia B

a. effect which pathway?
b. Effects on PT and PTT

A

a. Intrinsic pathway

b. Increased PTT, not effect on PT or INR

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17
Q

Symptoms of Hemophilia A/B

A

Macrohemorrhage –> hemarthroses, easy bruising, bleeding after trauma or surgery

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18
Q

Treatment for Hemophilia A, B and C

A

A - Desmopressin + factor 8 concentrate
B - factor 9 concentrate
C - factor 11 concentrate

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19
Q

Vitamin K Deficiency

a. Effects on PT, PTT
b. Mechanism

A

a. Increased PT, PTT
b. Decreased 10, 7, 9, 2
c. Vitamin K dietary deficiency, newborns (no gut bacteria), someone taking antibiotics, patients on Warfarin, end stage liver disease

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20
Q

Factor V Leidin

a. What does Factor V do?
b. Mechanism of disease

A

a. Factor V is a cofactor for factor 10 –> helps it activate thrombin
b. Factor V Leiden mutations makes Va resistant to inactivation by protein C –> increased coagulation

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21
Q

Prothrombin gene mutation (G20210A)

a. Mechanism
b. Effect

A

a. Mutation in 3’ intranslated region

b. Increased production of prothrombin –> increased plasma levels and venous clots

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22
Q

Antithrombin deficiency

a. Effect
b. PT/PTT

A

a. Unable to activate thrombin
b. No direct effect on PT, PTT or thrombin time but the increase in PTT is diminished after adding Heparin (reduced effect)

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23
Q

Protein C deficiency (or protein S)

A

Unable to activate factors 5 and 8 –> can’t shut off coagulation cascade –> increased risk of thrombotic skin necrosis with hemorrhage following administration of Warfarin

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24
Q

Skin and subcutaneous tissue necrosis after warfarin administration

A

Protein C deficiency

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25
Q

Mechanism of Heparin

A

Cofactor for activation of antithrombin –> decreased thrombin and factor 10a (only prevents clot from getting bigger or new clots from forming)
VERY SHORT HALF LIFE

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26
Q

Uses of Heparin

A

PE, acute stroke, MI, DVT

Can be used during PREGNANCY

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27
Q

What lab do we measure to monitor Heparin?

A

PTT

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28
Q

S/E of Heparin

A

Bleeding
Bone loss, osteoporosis
Heparin-Induced Thrombocytopenia (HIT) –> heparin binds to platelet factor 4 –> autoantibody complexes can then activate platelets to aggregate –> then they get removed from circulation and destroyed –> thrombosis and thrombocytopenia

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29
Q

What do you do for Heparin Induced Thrombocytopenia?

A

Stop Heparin

Start different anticoagulant like direct thrombin inhibitor (dabigatran, bivalirudin)

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30
Q

Direct Thrombin Inhibitors (specifically ones derived from Hirudin from leech spit)

A

Lepirudin
Bivalirudin
Desirudin

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31
Q

Direct Thrombin Inhibitors (NOT from hirudin)

A

Argatroban

Dabigatran

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32
Q

Low-molecular weight Heparins

A

Enoxaparin

Dalteparin

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33
Q

Advantages of low-molecular weight Heparins

Disadvantages?

A 
Better bioavailability
2-4 times longer half life
Administered SubQ
Don't need lab monitoring
Disadvantage - not easily reversible
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34
Q

Fondaparinux

A

Activates antithrombin to inhibit Factor Xa

Not a Heparin derivative

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35
Q

Mechanism of LMW Heparins?

A

Stimulate antithrombin to inactivate Factor Xa

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36
Q

Direct Factor 10a inhibitors

A

Rivaroxaban

Apixaban

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37
Q

Warfarin mechanism

A

Inhibits epoxide reductase –> interferes with gamma-carboxylation of vitamin K dependent clotting factors 2,7,9,10 Protein C and S
(Epoxide reducase recycles vitamin K)

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38
Q

Uses of Warfarin

A

Chronic antricoagulation –> Atrial fibrillation, DVT prophylaxis, DVT treatment, PE treatment

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39
Q

When is Warfarin C/I?

A

Pregnant patients - crosses placenta and is teratogenic

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40
Q

Toxicity of Warfarin

A

Bleeding, teratogen, skin/tissue necrosis, transient hyper coagulability

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41
Q

Reversal of Heparin

A

Protamine Sulfate

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42
Q

Reversal of Warfarin

A 
Vitamin K (oral) - takes days to do anything to reverse effects because have to wait for liver to make more clotting factors 
Fresh frozen plasma
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43
Q

Half life in Heparin vs. Warfarin

A

Short half life - Heparin

LONG half life - Warfarin

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44
Q

Onset of action in Heparin vs. Warfarin

A

Rapid (seconds) - Heparin

Slow - Warfarin

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45
Q

Thrombolytics

a. mechanism
b. Use

A

a. activated Plasmin –> lyses fibrin clot

b. EARLY ST elevation MI (< 3-6 hours), acute stroke, severe PE

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46
Q

S/E of thrombolytics

A

Bleeding (C/I in active bleeding, recent surgery, HTN, history of intracranial bleeding, coagulation defect)

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47
Q

Used to monitor coagulation in patient taking Warfarn

A

PT and INR

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48
Q

Characteristics of RBC

a. life span
b. shape
c. source of energy

A

a. 120 days
b. biconcave (spectrin), anucleate
c. Glucose 90% anaerobical metabolized to lactate, 10% used in HMP shunt

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49
Q

What is anisocytosis?

A

RBC of varying sizes

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50
Q

What is poikilocytosis?

A

RBC of varying shapes

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51
Q

What is a reticulocyte? What dose it reflect?

A

Immature RBC; reflects erythroid proliferation

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52
Q

What is polycythemia or erythrocytosis?

A

Too many RBCs

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53
Q

Basophilic stippling

Associated pathology?

A 
Little purple red dots (clumps of denatured RNA in basophil)
Lead poisoning (also thalassemias, anemia of chronic disease, alcohol abuse)
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54
Q

Echinocyte (burr cell)

A

Regular, uniform spikes all over surface

Seen in uremia, renal failure

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55
Q

Spurr cell (Acanthocyte)

A

IRREGULAR spiked cells
Liver disease
Abetalipoproteinemia

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56
Q

Spherocyte

A

RBCs that lose biconcave shape

-seen in Hereditary Spherocytosis

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57
Q

Schistocytes

A

Fragments of RBC that look like helmets
-Seen in DIC, TTP/HUS, HELLP, (pathologic intravascular clotting that RBCs are being forced through) from mechanical hemolysis

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58
Q

Target cells

A 
Look like bulls eye
THAL
Thalassemia
Hemoglobin C disease
Asplenia
Liver disease (also see acanthocytes)
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59
Q

Sickle cells

A

Crescent shaped

In sickle cell anemia

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60
Q

Howell Jolly body

A

Basophilic nuclear remnants found in RBCs
-Normally removed from RBCs by splenic macrophages; seen in patients with functional hyposplenia or asplenia (trauma, immune thrombocytopenia purpora splenic removal)

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61
Q

Heinz bodies

A

When hemoglobin gets oxidized and precipitates out of solution in RBC –> little clumps of Hgb

  • Spleen bites the Hgb clumps out of RBCs
  • Seen in G6PD deficiency (oxidative damage causes Heinz bodies)
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62
Q

How do you tell Heinz body from Howell Jolly Body?

A

Can only have ONE Howel jolly body but can have MANY Heinz bodies

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63
Q

Teardrop cells

A

Shaped like teardrops

-Seen with myelofibrosis

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64
Q

Elliptical shape cells (pencil cells)

A

Hereditary Elliptocytosis

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65
Q

Ring Sideroblasts

A

ABNORMAL

  • Seen in Bone marrow
  • Lead poisoning*** (big one), drugs, myelodysplastic syndromes
  • Sign of underlying diagnosis
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66
Q

Sideroblasts

A

Nucleated RBC precursor with granules of iron in mitochondria

  • Found in BM of healthy people
  • If you have disorder that affects heme synthesis and body can’t use all iron granules –> the granules surround and encircle the nucleus –> form ring
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67
Q

What happens if you transfuse incompatible blood type?

A

Antibody-mediated type II hypersensitivity

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68
Q

What is Erythroblastosis Fetalis?

A

Due to Rh incompatibility
Maternal Abs to fetal RBC antigens (Rh-D)
Rh- moms don’t have antibodies to Rh-D but after they are exposed to babies RBCs they form them (after first pregnancy)
-If babies blood comes into contact with babies blood in first pregnancy, mom’s immune system is sensitized to Rh- antigen and mom develops Abs –> then IgG can cross placenta and attack RBCs –> hemolysis –> Hgb breaks down into bilirubin and causes jaundice

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69
Q

Clinical features of Erythroblastosis Fetalis in infant

A

Anemia due to hemolysis of RBC by maternal Abs
Jaundice –> kernicterus possible
Hydrops fetalis (fetal edema)
Intrauterine death

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70
Q

Treatment of Erythroblastosis Fetalis

A

Administer RhoGAM (anti-Rh immunoglobulin) to Rh- pregnant women during 3rd trimester to prevent maternal anti-Rh IgG production (and any time there is trauma)

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71
Q

What kind of RBCs do you see in G6PD deficiency?

A

Bite cells

Heinz bodies

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72
Q

When do primitive blood cell progenitors appear in yolk sac?

A

at 3 weeks; come from mesonephros

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73
Q

Young Liver Synthesizes Blood

A 
Yolk Sac (week 3-8)
Liver (and spleen) 
Bone marrow (at 28 weeks)
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74
Q

Which part of skeleton is hematopoietically active?

A

Infancy and childhood - entire skeleton (sternum, pelvis, ribs, long bones, vertebrae, cranial)
Late adolescence, adulthood - vertebrae, sternum, ribs, pelvis

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75
Q

Fetal hemoglobin?

Adult hemoglobin?

A

Fetal - 2 alpha chains, 2 gamma chains (gamma has lower affinity for 2,3 DPG –> higher affinity for oxygen)
Adult - 2 alpha chains, 2 beta chains

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76
Q 
Hemoglobin variants
HbA
HbA2
HbA1c
HbF
HbGower
A 
HbA - 97% of normal (a2B2)
HbA2 - 2% of normal (a2delta2)
HbA1c - poorly controlled diabetes
HbF - fetal hemoglobin (a2gamma2)
HbGower - embryonic Hgb (2 zeta and 2 episilon chains)
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77
Q

HbS

A

Sickle cell Hgb
alpha 2, betaS2
Glu –> val in B chain

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78
Q

HbC

A

Hemoglobin C disease
alpha 2, betaC2
Glu –> lys in Beta chain

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79
Q

HbBart’s

A 
Severe alpha thalassemia
gamma 4 (no alpha chain)
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80
Q

HbH

A

Severe alpha thalassemia

B4 (no alpha chains)

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81
Q

What is rate limiting enzyme of heme synthesis?

What does it require?

A

aminolevulinic acid synthase (ALA synthase)
It makes glycine and succinyl-CoA into aminolevulinic acid
Requires Vitamin B6

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82
Q

What enzyme is defective in acute intermittent porphyria?

A

Porphobilinogen deaminase

Can’t make porphobilinogen into hydroxymethylbilane

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83
Q

Presentation of acute intermittent porphyria

A 
Abdominal pain (neuropathic)
Port wine- colored urine
Polyneuropathy
Psychological disturbanes
Precipitated by drugs (cytochrome p450 inducers, alcohol, starvation)
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84
Q

How do you treat acute intermittent porphyria?

A

Glucose and heme –> inhibit ALA synthase (no build up of neuro toxic products)

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85
Q

5 P’s of acute intermittent porphyria

A 
Pain in abdomen
Polyneuropathy
Port wine-colored urine
Psychological disturbances
Precipitated by drugs
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86
Q

What enzyme is defective in Porphyria Cutanea Tarda?

A

Uroporphyrinogen decarboxylase

Uroporphyrin accumulates and causes tea colored urine

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87
Q

Presentation of Porphyria Cutanea Tarda

A 
Most common form!
Blistering of skin and photosensitivity
Hypertrichosis
Facial hyperpigmentation
Tea-colored urine
Hepatitis C and alcoholism
Elevation of LFTs (AST, ALT)
(Think of homeless man)
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88
Q

How does lead poisoning affect heme synthesis pathway?

A

Inhibits Ferrochelatase –> Protoporphyin accumulates

Inhibits ALA dehydratase

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89
Q

Presentation of lead poisoning

A

Microcytic anemia (basophilic stippling) GI (abdominal colic) and Kidney disease
Mental deterioration in kids (also lead lines in bones, gingiva)
Headache, memory loss, demyelination in adults

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90
Q

Treatment of lead poisoning

A

EDTA or succimer to chelate lead

Dimercaprol + succimer in kids for very severe lead poisoning

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91
Q

What is Polycythemia vera?

A

Monoclonal proliferation of RBCs

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92
Q

Causes of polycythemia vera

A

Chronic hypoxia –> to increase O2 carrying capacity (pulmonary disease, cyanotic heart disease, high altitudes)
Tumors
Trisomy 21 at birth

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93
Q

Tumors that cause polycythemia vera

Potentially Really High Hematocrit

A

Pheochromocytoma
Renal cell carcinoma
Hepatocellular carcinoma
Hemangioblastoma

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94
Q

Relative Polycythemia

A

Plasma volume is reduced so that red cell count is increased relative to plasma volume

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95
Q

Absolute polycythemia

A

Plasma volume is normal and you have too many RBCs

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96
Q

Appropriate absolute polycythemia

a. plasma volume
b. RBC mass
c. O2 saturation
d. EPO levels
e. associations

A

a. no change
b. Increased
c. decreased
d. increased
e. seen in lung disease, congenital heart disease, high altitude

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97
Q

Inappropriate absolute polycythemia

a. plasma volume
b. RBC mass
c. O2 saturation
d. EPO levels
e. associations

A

a. no change
b. Increased
c. no change
d. increased
e. renal cell carcinoma, hepatocellular carinoma, hydronephrosis

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98
Q

Polycythemia vera

a. plasma volume
b. RBC mass
c. O2 saturation
d. EPO levels
e. associations

A

a. increased
b. really increased
c. no change
d. decreased
- EPO decreased due to negative feedback suppressing renal EPO production

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99
Q

Relative polycythemia

a. plasma volume
b. RBC mass
c. O2 saturation
d. EPO levels
e. Associations

A

a. decreased
b. no change
c. no change
d. no change
e. Decreased plasma volume like dehydration and burns

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100
Q

Microcytic Anemia

A 
Iron Deficiency Anemia 
Alpha thalassemia
Beta thalassemia
Lead poisoning
Sideroblastic anemia 
Anemia of Chronic Disease
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101
Q

Iron Deficiency Anemia - causes

A 
Poor intake
Blood loss (menstruation or GI chronic occult blood loss)
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102
Q

IDA - characteristics

A

Hypochromic

Microcytic

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103
Q

What is Plummer Vinson syndrome?

A

Iron deficiency anemia
Esophageal web
Atrophic glossitis

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104
Q

Alpha Thalassemia - cause

Affects who?

A

Defect in alpha globin gene deletions –> defect in alpha globin synthesis
African/Asian populations

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105
Q

alpha thalassemia

a. mutation of one allele
b. mutation in 2 alleles
c. mutate 3 alleles
d. mutate 4 alleles

A

a. no anemia
b. alpha thal trait, no anemia
c. Hemoglobin H disease (beta globins pair up with each other )
d. incompatible with life - Hemoglobin Barts - four gamma globes –> causes hydrous fetalis and death

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106
Q

Beta thalassemia cause

Affects who?

A

Defect in beta globin gene (point mutation) –> decreased beta globin synthesis
Mediterranean populations

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107
Q

B-thalassemia minor (heterozygote)

A

B chain is underproduced
Usually asymptomatic
Diagnosis confirmed by increased HbA2 (>3.5%) on electrophoresis

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108
Q

B-thalassemia major (homozygote)

A

B chain is absent –> severe anemia requiring blood transfusion
Marrow expansion (crew cut on skull X-ray) –> skeletal deformities, chipmunk facies
Extramedullary hematopoiesis leads to hepatosplenomegaly
-Increased risk of parvovirus B19 induced aplastic crisis

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109
Q

Why do you have to confirm diagnosis of iron deficiency in patients with microcytic anemia before you start iron supplements?

A

They might have beta thalassemia in which case they would have increased iron from thalassemia

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110
Q

Peripheral smear of thalassemia shows…

A

Target cells

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111
Q

What are two enzymes in heme synthesis that are affected by lead poisoning?

A

Ferrochelatase

ALA dehydratase

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112
Q

Causes of sideroblastic anemia

A

Lead poisoning
Alcohol
Drugs (seizure drugs, Rifampin)
Hereditary X linked defect in ALA synthase

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113
Q

Anemia of chronic disease

Iron, Ferritin levels

A

From defective iron utilization
Iron is trapped in macrophages so serum iron is LOW
Ferritin is normal or HIGH

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114
Q

Macrocytic anemia

a. Megaloblastic anemia
b. Non-megaloblastic anemia

A

a. B12 deficiency, Folate deficiency , orotic aciduria

b. Liver disease, Alcoholism, Reticulocytes

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115
Q

RBC appearance in B12/folate deficiency

A

Hypersegmented neutrophils (>6 lobes)

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116
Q

Causes of B12 deficiency

A 
Insufficient intake
Malabsorption (Crohn, removal of terminal ileum)
Pernicious anemia
Diphyllobothrium latum (fish tapeworm)
Gastrectomy
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117
Q

Causes of folate deficiency

A

Malnutrition (alcoholics)
Malabsorption
Drugs (methotrexate, phenytoin, trimethoprim)
Increased requirement (pregnancy, hemolytic anemia)

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118
Q

Findings in

a. Folate deficiency
b. B12 deficiency

A

a. NO NEUROLOGIC SYMPTOMS; increased homocysteine, normal methylmalonic acid; megaloblastic anemia
b. Neurologic symptoms (subacute combined degeneration, spinocerebellar tract, lateral corticospinal tract, dorsal column dysfunction, dementia, neuropathy); Methylmalonic acid INCREASED, increased homocysteine

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119
Q

Why does B12 deficiency cause subacute combined degeneration?

A

B12 is involved in fatty acid pathways and myelin synthesis

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120
Q

Why should you not treat with empiric folic acid for megaloblastic anemia?

A

It might fix anemia but if B12 is deficient it will NOT correct neurologic deficits

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121
Q

What is orotic aciduria?

A

Defect in UMP synthase (pyrmidine synthase)
Causes orotic acid in urine, megaloblastic anemia Developmental delay
Failure to thrive
NO hyperammonemia (vs. ornithine transcarbamylase deficiency)

122
Q

NON-megaloblastic macrocytic anemia cause

A 
DNA synthesis is NOT impaired
Alcoholism
Liver disease
Hypothyroidism
Reticulocytosis
Drugs (5-FU, Zidovudine, Hydroxyurea)
123
Q

Normocytic NON-hemolytic anemia causes

A

Anemia of chronic disease

Aplastic anemia

124
Q

Anemia of chronic disease

a. cause
b. Iron, TIBC, Ferritin levels

A

a. Lots of inflammation causes lots of inflammatory mediators like Hepcidin to be increased –> inhibits iron transport because iron is trapped in macrophages
b. Decreased iron, decreased TIBC, Increased Ferritin

125
Q

Conditions associated with anemia of chronic disease

A

SLE, RA, Neoplastic disorders, CKD

126
Q

Aplastic anemia

a. causes
b. Findings
c. symptoms

A

a. Radiation***** and drugs, Viral agents (parvovirus B19, EBV, HIV, HCV), Fanconi anemia (DNA repair defect), Idiopathic (immune mediated)
b. Pancytopenia (anemia, leukopenia, thrombocytopenia)
Normal cell morphology but HYPOCELLULAR BONE MARROW With fatty infiltration
c. Fatigue, malaise, pallor, purpura, petechiae, infection

127
Q

Treatment of aplastic anemia

A

Withdrawal of offending agent
Bone marrow allograft
RBC/platelet transfusion
Bone marrow stimulation

128
Q

What test can be used to diagnose Beta thalassemia minor?

A

Hemoglobin electrophoresis to look for higher than normal levels of HbA2

129
Q

Megaloblastic anemia not correctable by B12 or Folate

A

Orotic Aciduria

130
Q

Microcytic anemia reversible with B6

A

Sideroblastic anemia

131
Q

Drugs that can cause sideroblastic anemia

A

Isoniazid (inhibits B6)
Lead
Copper deficiency
B6 deficiency

132
Q

HIV patient with macrocytic anemia

A

Zidovudine

133
Q

Normocytic anemia with elevated creatinine

A

Chronic kidney disease resulting in low EPO

134
Q

Skull x-ray shows “hair on end” appearance

A

Result of marrow hyperplasia (crew cut)

135
Q

Basophilic stippling of RBCs

A

Lead poisoning

136
Q

Co-factor required for pyruvate dehydrogenase
Tender Loving Care For No-one
What other enzyme requires these?

A 
Thiamine
Lipoid acid
Coenzyme A
FAD
NAD
-Also required by Alpha-ketoglutarate dehydrogenase
137
Q

Intravascular or Extravascular hemolysis?

a. Decreased serum haptoglobin
b. Increased LDH
c. Increased unconjugated bilirubin

A

a. Massive intravascular hemolysis causes haptoglobin to bind all free hemoglobin
b. released from RBCs (intravascular OR extravascular hemolysis)
c. Seen in extravascular hemolysis (with intravascular all the heme would be bound by haptoglobin)

138
Q

Intravascular hemolysis Causes

A

Autoimmune hemolytic anemia
Paroxysmal nocturnal hemoglobinuria
Mechanical destruction of RBCs

139
Q

Extravascular hemolysis

Causes

A

Hereditary spherocytosis (spleen recognizes abnormal cells)
G6PD Deficiency (extravascular AND intravascular)
Pyruvate kinase deficiency
Sickle cell
Hemoglobin C disease

140
Q

Hereditary spherocytosis defect

A

Defect in Ankyrin and spectrin (proteins interacting with RBC membrane skeleton and plasma membrane) –> red cells are small round spheres with NO central pallor and less surface area

141
Q

Hereditary spherocytosis

a. Increased MCHC - why?
b. increased RDW - why?
c. Why does it cause hemolysis

A

a. Increased Mean corpuscular hemoglobin content; same amount of Hgb but they are smaller so it is more concentrated
b. Not as much variability with red cell size
c. Spleen recognizes cells as normal and removes them –> splenomegaly

142
Q

Diagnostic test for Hereditary spherocytosis

A

Osmotic fragility test (high percentage of lysis in NaCl)

143
Q

Treatment for hereditary spherocytosis

A

Splenectomy (so that it doesn’t remove red cells) –> then you see Howell Jolly bodies

144
Q

G6PD deficiency defect

A

X linked recessive

Defect in G6PD –> causes decreased glutathione and increased RBC susceptibility to oxidative stress

145
Q

Stressors that cause anemia in G6PD deficiency

A

Fava beans
Sulfa drugs
Anti-malarials
Infections

146
Q

Symptoms of G6PD deficiency

A

Back pain, hemoglobinuria a few days after oxidant stress

147
Q

Pyruvate kinase deficiency causes…

A

Intrinsic hemolytic anemia
Defect in pyruvate kinase –> decreased ATP –> rigid RBCs
Hemolytic anemia in newborn

148
Q

Paroxysmal Nocturnal Hemoglobinuria defect

A

Red cells missing surface markers CD55 and CD59 so complement attacks them and lyses them

149
Q

What is test for PNH?

A

Ham’s test - add acid to lower pH and that activates complement and lyses RBCs

150
Q

Triad of PNH

A

Coombs - hemolytic anemia
Pancytopenia
Venous thrombosis

151
Q

Sickle cell disease defect

A

Hemoglobin S mutation is single aa replacement where valine is used instead of glutamic acid at position 6 –> allow Hgb to polymerize in RBC

152
Q

What triggers sickling in SCD?

A

Hypoxemia
Dehydration
Acidosis

153
Q

Complications of Sickle Cell Disease

A

Aplastic crisis
Autosplenectomy
Splenic infarct
Salmonella osteomyelitis
Painful crises (vaso-occlusive)–> acute chest syndrome, avascular necrosis, stroke, dactylitis
Renal papillary necrosis and microhematuria

154
Q

What organism causes osteomyelitis in SCD?

A

Salmonella

155
Q

Treatment for SCD

A

Hydroxyurea

BM transplantation

156
Q

Hemoglobin C disease defect

A

Glutamic acid–> Lysine mutation in B globin

157
Q

Microangiopathic anemia cause

A

RBCs are mechanically damaged as they pass through lumen of obstructed vessel –> in DIC, TIP/HUS, lupus, malignant HTN –> RBC gets chopped up by shear force or fibrin strands

158
Q

Macroangiopathic anemia cause

A

Damage to cells by forces in LARGER vessels (prosthetic heart valves, aortic stenosis)

159
Q

Autoimmune Hemolytic Anemia a. Warm agglutinins

b. Cold agglutinins

A

a. IgG Abs attach to red cells and cause them to agglutinate AT BODY TEMP
b. IgM Abs that only cause problems when temp is lower

160
Q

Cold agglutinins occur in…

A

Infections with EBV, mycoplasma

Malignancies (CLL)

161
Q

Warm agglutinins occur in…

A 
SLE
Malignancies (CLL)
Drugs (methyldopa)
Viruses (EBV, HIV)
Congenital immune abnormality
162
Q

Direct Coombs Test

A

Anti-Ig antibody (Coombs reagent) is added to patient’s blood –> the RBCs agglutinate if RBCs are coated with Ig

163
Q

Indirect Coombs Test

A

Normal RBCs added to patient’s serum –> if serum has anti-RBC surface Ig, RBCs agglutinate when Coombs reagent is added

164
Q

Direct Coombs test is positive in:

A

Hemolytic disease of newborn
Drug-induced autoimmune hemolysis anemia
Hemolytic transfusion reactions

165
Q

Indirect Coombs test positive when:

A

Abs present to foreign blood (test blood prior to transfusion, screen maternal antibodies to a fetus’ blood)

166
Q

Painful cyanosis of fingers and toes with hemolytic anemia

A

Cold autoimmune hemolytic anemia

167
Q

Red urine in the morning, and fragile RBCs

A

PNH

168
Q

Basophilic nuclear remnants in RBCs

A

Howell-Jolly bodies

169
Q

Autosplenectomy

A

Sickle cell (from auto-infarcting)

170
Q

Drug used to treat sickle cell disease

A

Hydroxyurea

171
Q

Where do platelets come from?

A

Megakaryocytes

172
Q

How long do platelets live?

A

8-10 days; have no nucleus

173
Q

What is vWF? Where does it come from?

What is it’s function?

A

It comes from Weibel-Palade bodies of endothelial cells and alpha granules of platelets
It complexes with and stabilizes factor 8 (elevated PTT with deficiency)
It binds to exposed collagen and then platelets binds vWF via Gp2b receptor at site of injury

174
Q

How do platelets become activated after adhesion?

A

They change shape and ADP binding to receptor induces Gp2b/3a expression at platelet surface

175
Q

How do platelets aggregate after activation?

A

Fibrinogen binds Gp2b/3a and link platelets

176
Q

Pro-aggregation of platelet factors

A

TXA2 (released by platelets)
Decreased blood flow
Increased platelet aggregation

177
Q

Anti-aggregation of platelet factors

A

PGI2 and NO (endothelial cells)
Increased blood flow
Decreased platelet aggregation

178
Q

Aspirin mechanism

A

Inhibits cyclooxygenase –> permanently blocks the conversion of arachidonic acid to thromboxane A2

179
Q

Effects of Aspirin on bleeding, PT, PTT

A

Increased bleeding time

No effect on PT, PTT

180
Q

Uses of Aspirin

A

Reduce fever
Treat pain
Anti-inflammatory
Anti-platelet

181
Q

S/E of Aspirin

A

Bleeding
Peptic ulcers (no protective prostaglandins)
Hyperventilation (stimulates respiratory centers of brain) –> respiratory alkalosis (but transitions to metabolic acidosis)
Reye’s syndrome (Hepatoencephalopathy)

182
Q

Mechanism of Clopidogrel

A

ADP receptor inhibitor (inhibits platelet aggregation by preventing expression of GP2b/3a)

183
Q

Uses of Clopidogrel

A

ACS, Coronary stenting

184
Q

2b/3a INhibitors

A

Abciximab
Eptifibatide
Tirofiban

185
Q

ADP receptor blockers

A

Clopidogrel
Prasugrel
Ticagrelor (reversible)
Ticlopidine (causes neutropenia)

186
Q

Uses for Gp2b/3a inhbitors

A

NSTEMI
Acute coronary syndrome
unstable angina
Percutaneous transluminal coronary angioplasty

187
Q

Platelet abnormalities symptoms

A 
Mucous membrane bleeding
Epistaxis
Petechiae 
Purpura
Increased bleeding time
188
Q

Immune Thrombocytopenia

a. mechanism
b. Signs
c. BM biopsy shows…

A

a. Autoimmune disease with Abs to Gp2b/3a –> immune system removes and destroys them
b. Platelet count is low
c. Increased megakaryocytic on BM biopsy

189
Q

Treatment for ITP

A

Steroids
IV immunoglobulin
Splenectomy

190
Q

Thrombotic thrombocytopenia purpura
a. mechanism
b. signs/symptoms
c.

A

a. platelets are excessively activated because of increased large vWF multimers –> widespread thrombosis –> uses up all the platelets and causes thrombocytopenia and bleeding
b. pentad of neurologic and renal symptoms: fever, thrombocytopenia, microangiopathic hemolytic anemia

191
Q

Defect in TTP

A

Inhibition or deficiency of ADAMTS13 (metalloproteases) that cleaves and degrades vWF multimers

192
Q

Treatment for TTP

A

Plasmapheresis

Steroids

193
Q

What condition is TTP closely linked to?

A

Hemolytic Uremic Syndrome

194
Q

What is triad of findings in HUS?

A

Microangiopathic hemolytic anemia
Renal symptoms
Thrombocytopenia

195
Q

Five features of TTP/HUS

Nasty Fever Torched His Kidneys

A 
Neurologic
Fever
Thrombocytopenia
Hemolysis
Kidney failure
196
Q

What is HUS associated with in kids?

A

E. coli 0157H7

197
Q

Bernard Soulier syndrome defect

A

Defect in glycoprotein 1b –> platelet can’t bind collagen –> defect of platelet plug formation from defect in adhesion

198
Q

Glanzmann thrombasthenia defect

A

Defect in Gp2b/3a –> defect in platelet aggregation –> platelet count normal, bleeding time prolonged

199
Q

von Willebrand disease

a. defect
b. causes

A

a. defect in vonWillebrand factor
b. defect in platelet plug formation from defect in adhesion AND intrinsic pathway coagulation defect
c. Increased PTT and increased bleeding time

200
Q

Treatment of vWF disease

A

Desmopressin (increased release of vWF in storage sites of endothelial cells)

201
Q

Disseminated Intravascular Coagulation

a. mechanism

A

a. widespread activation of clotting –> deficiency in clotting factors and platelets –> bleeding state

202
Q

Causes of DIC

STOP Making New Thrombi

A 
Sepsis
Trauma
Obstetric complications!!!!!
acute Pancreatitis
Malignancy
Nephrotic syndrome
Transfusion
203
Q

DIC

a. PT and PTT
b. Bleeding time
c. Fibrinogen
d. D-dimer

A

a. Prolonged
b. increased bleeding time
c. low fibrinogen
d. high fibrin split products (d-dimer)

204
Q

peripheral smear of DIC

A

Schistocytes

205
Q

Presentation of DIC

A

Multi organ failure

Bleeding

206
Q

Most common inherited bleeding disorder

A

vWF disease

207
Q

What does leukemia affect?

What does lymphoma affect?

A

Bone marrow, maybe peripheral blood

Lymphoma is tumor in lymph nodes

208
Q

What makes lymphoma a Hodgkin lymphoma?

A
  • Presence of Reed Sternberg cells (bilobed nuclear, clearing around nuclei, owl eye appearance, clearing around cell from cell shrinking)
  • Found in single group of lymph nodes (extra nodal involvement is rare)
  • Painless, nontender lymphadenopathy (nodes feel firm and rubbery)
  • Lymphadenopathy in mediastinum
  • Constitutional B symptoms: Low grade fever, night sweats, weight loss
209
Q

Who gets Hodgkin lymphoma?

A
  • Bimodal age (age 20 and 65)

- More common in men (except nodular sclerosing)

210
Q

What are most non-Hodgkin lymphomas? Age distribution?

A

B cell lymphomas

Age distribution is widely variable

211
Q

How do symptoms of non-Hodgkin compare to Hodgkin?

A

Fewer constitutional symptoms

212
Q

What are certain types of non-Hodgkin lymphoma associated with?

A

HIV and immunocompromised

213
Q

How do Reed Sternberg cells relate to prognosis?

A

The fewer cells the better prognosis

214
Q

What is the most common type of Hodgkin lymphoma?

A

Nodular sclerosing type

215
Q

Which type of Nodular sclerosing Hodgkin lymphoma has the best prognosis?

A

Lymphocyte rich form

Lymphocyte mixed or depleted form has worst prognosis

216
Q

What are cell markers of Reed Sternberg cells?

A

CD15+

CD30+

217
Q

Most common type of NH lymphoma in adults

A

Diffuse large B cell lymphoma

218
Q

Translocation in diffuse large B cell lymphomas

A

t(14:18)

219
Q

Follicular lymphoma translocation

A

t(14;18) of heavy chain Ig (14) and BCL-2 (18) –> BCL2 inhibits apoptosis

220
Q

Burkitt lymphoma translocation

A

t(8;14) –> translocation of c-myc and heavy chain Ig

221
Q

Mantle cell lymphoma translocation

A

t(11;14) –> translocation of cyclin D1 and heavy chain Ig

222
Q

Starry sky appearance (sheets of lymphocytes with interspersed macrophages)

A

Burkitt lymphoma

223
Q

Associated with EBV

A

Burkitt lymphoma

224
Q

Endemic form of Burkitt lymphoma

A

Jaw lesion

225
Q

Indolent course of lymphoma; painless waxing and waning lymphadenopathy

A

Follicular lymphoma

226
Q

Associated with Sjogrens, Hashimotos, and H. pylori

A

Marginal cell MALToma

227
Q

Adults present with cutaneous lesions (Japan, West African, Caribbean)

A

Adult T cell Lymphoma

228
Q

Lytic bone lesions, Hypercalcemia

A

Adult T cell Lymphoma

229
Q

Caused by HTLV (associated with IV drug abuse)

A

Adult T cell Lymphoma

230
Q

Presents with skin patches/plaques characterized by atypical CD4 cells with cerebriform nuclei

A

Mycosis fungoides

231
Q

If mycosis fungoides progresses to blood

A

Sezary syndrome

232
Q

Associated with long term celiac disease

A

Intestinal T cell lymphoma

233
Q

Lymphoma equivalent of CLL

A

Small lymphocytic lymphoma

234
Q

Cancer most commonly associated with a non-infectious fever

A

Hodgkin lymphoma

235
Q

Large B cells with bilobed nuclei and prominent owl’s eye inclusions

A

Reed Sternberg Cells seen in Hodgkin Lymphoma

236
Q

Large B cells with bilobed nuclei and prominent owl’s eye inclusions

A

Reed Sternberg Cells seen in Hodgkin Lymphoma

237
Q

What’s the difference between acute and chronic leukemia?

A

Acute - rapid onset and rapidly progressive, >50% blasts on BM biopsy, (myeloblasts in AML, Lymphoblasts in ALL), numerous blast cells (>20% blasts), often associated with pancytopenia
Chronic -

238
Q

ALL is most common in…

A

Children Female

Whites > Blacks

239
Q

Common presentation of T-ALL

A

Mediastinal mass presenting as SVC like syndrome

240
Q

Prognosis of ALL

A

Good prognosis in children (up to 90% remission)

241
Q

TdT+, CD10+

A

TdT+ is a marker of preT and preB cells

CD10+ is markers of preB cells

242
Q

ALL translocation that equals better prognosis

A

t(12;21)

243
Q

ALL association

A

Down syndrome

244
Q

Common ALL symptom

A

Bone pain

245
Q 
ALL association
(We all fall down)
A

Down syndrome

246
Q

Common ALL symptom

A

Bone pain

247
Q

Age of onset for AML

A

median onset 65 years

248
Q

AML blood smear shows:

A 
Auer rods (peroxidase cytoplasmic inclusions seen in M3 AML)
Increased circulating myeloblasts
249
Q

Risk factors for AML

A

Prior exposure to alkylating chemotherapy, radiation, myeloproliferaetive disorders, Down syndrome

250
Q

t(15;17)

A

M3 AML subtype responds to all-trans retinoid acid (vitamin A), inducing differentiation of myeloblasts (Acute promyelocytic AML)

251
Q

DIC common presentation of:

A

M3 AML

252
Q

CD13/33+

A

AML

253
Q

PAS (-)

A

AML

254
Q

What’s the difference between acute and chronic leukemia?

A

Acute - rapid onset and rapidly progressive, >50% blasts on BM biopsy, (myeloblasts in AML, Lymphoblasts in ALL), numerous blast cells (>20% blasts), often associated with pancytopenia
Chronic - Insidious onset and gradual progression (months to years), mature cells (

255
Q

PAS (-)

A

AML

256
Q

Most common adult leukemia seen in western countries

A

CLL

257
Q

Who does CLL affect?

A

Adults over age 50

258
Q

95% have B cell markers

A

CLL

259
Q

Smudge cells

A

CLL

260
Q

Autoimmune hemolytic anemia

A

CLL

261
Q

Autoimmune hemolytic anemia

A

CLL

262
Q

CD20+, CD5+ B cell Neoplasm

A

SLL/CLL

263
Q

Defined by the Philadelphia chromosome t(9;22), BCR-ABL

A

CML

264
Q

Presents with increased neutrophils, metamyelocytes, basophils; splenomegaly

A

CML

265
Q

Responds to Imatinib

A

CML

266
Q

Very low LAP as a result of low activity in mature granulocytes

A

(vs. Leukemoid reaction which has Increased LAP)

CML

267
Q

Autoimmune hemolytic anemia

warm or cold agglutinins

A

CLL

268
Q

Presents with increased neutrophils, metamyelocytes, basophils; splenomegaly, fatigue, abdominal pain

A

CML

269
Q

Very low LAP as a result of low activity in mature granulocytes

A

(vs. Leukemoid reaction which has Increased LAP)

CML

270
Q

Age of presentation of CML

A

25-60

271
Q

a. CLL progress to ALL?

b. CML progress to AML?

A

a. 10% progress to ALL

b. 80% progress to AML; this is Blast crisis

272
Q

What is Philadelphia chromosome?

A

t(9;22) BCR ABL mutation

Encodes constitutively activated tyrosine kinase –> malignant transformation

273
Q

What is Philadelphia chromosome?

A

t(9;22) BCR ABL mutation

Encodes constitutively activated tyrosine kinase –> malignant transformation

274
Q

t(8;14)

A

Burkitt lymphoma

275
Q

t(15;17)

A

M3 AML (auer rods); treated with ATRA

276
Q

t(8;21)

A

AML

This is why patients with Trisomy 21 have increased incidence of AML.

277
Q

What is Leukemoid reaction?

A 
Increased WBC as a reaction to a stressor
Predominantly neutrophils
Left shift (5-10% immature bands)
Increased LAP (CML has decreased LAP)
278
Q

What is Myelodysplastic syndrome?

A

Dysplasia of the hematopoietic cells in the myeloid tissue

279
Q

What is Pseudo-Pelger Huet anomaly?

A

Neutrophils with bilobed nuclei; seen after chemotherapy

280
Q

What are myeloproliferative disorders?

A

Neoplastic transformation of a single myeloid precursor –>
Monoclonal proliferation of mature myeloid cells
Polycythemia vera
Essential thrombocytosis
Myelofibrosis

281
Q

Mutation common in myeloproliferative disorders

A

JAk2 –> encodes Janus kinase 2 –> switched on all the time –> constantly getting growth factor signal to make more cells

282
Q

What is Polycythemia vera? What is the mutation?

A

Jak2 mutation in red cell precursor; causes increased hematocrit without elevated EPO

283
Q

Presentation of polycythemia vera

A

Intense itching after hot shower (from increased basophils)
Erythromelalgia (severe, burning pain and red-blue coloration) from episode blood clots in vessels of extremities
Plethora (redness of face)
Hyper viscosity of blood
Splenomegaly
Headache

284
Q

What is essential thrombocytosis

A

Overproduction of abnormal platelets –> bleeding, thrombosis

285
Q

What does BM look like in Essential thrombocytosis?

A

Has enlarged megakaryocytes

286
Q

What is myelofibrosis?

A

Fibrosis and obliteration of the marrow space

Causes teardrop red cells

287
Q

What is myelofibrosis?

A

Fibrosis and obliteration of the marrow space

Causes teardrop red cells

288
Q

What is multiple myeloma?

A

Monoclonal plasma cell cancer that arises in marrow and produces lots of IgG (55%) or IgA (25%)

289
Q

Most common primary tumor arising within bone in people >40-50

A

Multiple myeloma

290
Q

Most common primary tumor arising within bone in people >40-50

A

Multiple myeloma

291
Q

How do you identify plasma cells?

A

Clock face chromatin and intracytoplasmic inclusions containing immunoglobulin

292
Q

Presentation of multiple myeloma

A

Anemia (plasma cells interfere with production of other cells in BM)
Renal insufficiency (form casts and plug up kidneys)
Back Pain (bc they secrete ostend cytokines) Punched out lytic bone lesions!
Hypercalcemia
Susceptibilty to infection (decreased production of healthy immune cells)
Amyloidosis

293
Q

Serum protein electrophoresis for Multiple myeloma shows:

A

Monoclonal antibody (M) spike

294
Q

Bence Jones proteins

A

Immunoglobulin light chains in urine detected on urine protein electrophoresis
BUT Do NOT see elevated protein on regular urinalysis

295
Q

Peripheral smear of multiple myeloma

A

Rouleaux formation (RBCs stacked like poker chips in bloos smear)

296
Q

Peripheral smear of multiple myeloma

A

Rouleaux formation (RBCs stacked like poker chips in bloos smear)

297
Q

What is Waldenstrom macroglobulinemia?

A

Shows M spike that is IgM
Hyperviscosity syndrome and Amyloidosis (blurred vision, Raynaud phenomenon)
No CRAB findings

298
Q

Plasmacytoma

A

2 types: solitary plasmacytoma of bone and extra medullary plasmacytoma (predilection for head and neck -specifically nose)

299
Q

What is Monoclonal Gammopathy of Undetermined significance?

A

Monoclonal expansion of plasma cells, asymptomatic, may lead to multiple myeloma
Need to be monitored

300
Q

What is Monoclonal Gammopathy of Undetermined significance?

A

Monoclonal expansion of plasma cells, asymptomatic, may lead to multiple myeloma
Need to be monitored

301
Q

Most common leukemia in children

A

ALL

302
Q

Acute leukemia positive for peroxidase

A

AML

Step 1 AB (29 decks)

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