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Flashcards in Heme/Onc Random Facts Deck (72):
1

What is the life span of a thrombocytes.

8-10 days

2

What are the granules in platelets?

Dense granules-contain ADP and Ca, alpha-granules contain vWF and fibrinogen

3

Where is 1/3 of the platelet pool stored

In the spleen

4

What are the granules in neutrophils?

Specific granules contain ALP, collagenase, lysozyme, and lactoferrin. Azurophilic granules (lysosomes) contain proteases, acid phosphatase, myeloperoxidase, and beta-glucuronidase

5

How are macrophages involved in septic shock?

Lipid A from bacterial LPS binds CD 14 on macrophages to initiate septic shock

6

What activates macrophages?

Gamma-interferon

7

What does eosinophilic mean?

Pink

8

What does basophilic mean?

Blue/purple

9

What are the causes of eosinophilia?

Neoplasia, asthma, allergy, chronic adrenal insufficiency, parasites

10

What die the granules in basophils contain?

Heparin and histamine. Leukotrienes synthesized and released on demand

11

What can basophilia be a sign of?

Myeloproliferative disease, particularly CML

12

What's the universal donor and universal recipient of plasma?

AB is the universal plasma donor (because no antibodies in their plasma), O is the universal recipient of plasma (because antibodies have nothing to react with)

13

How does warfarin work?

It inhibits vitamin K episode reductase, which is needed to activate the vitamin K dependent clotting factors

14

MOA of antithrombin

Inhibits activated forms of factors 2, 7, 9, 10, 11, and 12. Principle targets of antithrombin: thrombin and factor Xa

15

What does tPA do?

Converts plasminogen to plasmin

16

MOA of protein C and S

Protein C is converted to activated protein C by thrombin-thrombin insulin complex on endothelial cells, APC is activated by protein S to cleave and inactivate factor Va and VIIIa

17

MOA of clopidogrel, prasugrel, and ticlipidine

Inhibit ADP-induced expression of GpIIb/IIIa

18

MOA of abciximab, eptifibatide, and tirifiban

Inhibit GpIIb/IIIa directly

19

What is Bernard Soulier syndrome

A deficiency of GpIb

20

What is Glazmann thrombasthenia

Deficiency of GpIIb/IIIa

21

When might you see a failure of agglutination with ristocetun assay?

Von Willebrand disease and Bernard-Soulier syndrome

22

When might you see acanthocytes ("spur cells")

Liver disease, abetalipoproteinemia

23

When might you see target cells

HbC disease, Asplenia, liver disease, thalassemia

24

What are Heinz bodies?

Oxidation do Hb-SH groups to -S--S- causing hemoglobin precipitation with subsequent damage to RBC membrane causing bite cells

25

What might you see in a reticulocyte in Wright-Giemsa stain

Blue staining, represents the residual ribosomal RNA

26

Where does iron absorption occur?

In the duodenum and proximal jejunum so bypassing this in a gastrojejunostomy results in iron deficiency anemia

27

What is associated with koilonychia?

(Spoon nails) iron deficiency anemia

28

What happens with a four allele deletion in the alpha-globin gene?

Hemoglobin Barts (4 Gamma chains) forms. Is incompatible with life

29

What is hemoglobin H?

Four beta chains

30

What ethnicities are associated with alpha and beta thalassemia?

Alpha- Asian and African, beta-Mediterranean

31

How is beta thalassemia minor diagnosed?

Increased HbA2 on electrophoresis

32

MOA of lead poisoning

Inhibits ferrochatase and ALA dehydratase causing decreased heme synthesis and increased RBC protoporphyrin

33

Signs and symptoms of lead poisoning

Lead lines on gingivae and metaphysics of long bones on xray, encephalopathy and erythrocytes basophilic stippling, abdominal colic and sideroblastic anemia, drops-wrist and foot

34

What causes hereditary sideroblastic anemia?

X-linked defect in delta-ALA synthase gene

35

Causes of sideroblastic anemia

Genetic, acquired (myelodysplastic syndromes) and reversible (alcohol, lead, vitamin B6 deficiency, copper deficiency, isoniazid)

36

What is orotic aciduria?

AR defect in UMP synthase, presents as FTT, developmental delay, and megaloblastic anemia refractory to folate and B12. NO hyperammonemia (vs ornithine transcarbamylase deficiency)

37

Tx of orotic aciduria

Uridine monophosphate to bypass mutated enzyme

38

What might have an abnormal eosin-5-maleimide binding test

Hereditary spherocytosis

39

Pyruvate kinase deficiency

AR, causes decreased ATP causing rigid RBCs and extra vascular hemolytic anemia. Hyperplasia of the red pulp of the spleen

40

Mutation in HbC disease

Glutamic acid to lysine in beta-globin, causes extravascular hemolysis

41

Triad, labs, and tx in PNH

Triad: Coombs - hemolytic anemia, pancytopenia, and venous thrombosis, labs: CD55/59 neg RBCs on flow cytometry, tx: eculizumab

42

Warm agglutinin

Chronic anemia seen in SLE and CLL and with certain drugs (alpha-methyldopa), IgG

43

Cold agglutinin

IgM, acute anemia triggered by cold, seen in CLL, mycoplasma and infectious mononucleosis

44

What is the direct Coombs test

Anti-Ig antibody added to patient's blood. RBCs agglutinate if RBCs are coated with Ig

45

Indirect Coombs

Normal RBCs added to patients serum. If serum has anti-RBC surface Ig, RBCs agglutinate when Coombs reagent is added

46

Effect of corticosteroids on neutrophils, eosinophils, and lymphocytes

Neutrophilia from demargination, eosinopenia from sequestration in lymph nodes, lymphopenia from apoptosis

47

Enzyme inhibited in acute intermittent porphyria

Porphibiligen deaminase

48

Enzyme deficient in porphyria Cutanea tarda

Uropirphyrinogen decarboxylase

49

Pathogenesis of ttp

Inhibition or deficiency of ADAMTS13 (vWF metalloprotease) causes large vWF multimers which causes increased platelet adhesion, which causes platelet aggregation and thrombosis

50

Pentad of ttp

Neurologic and renal sx, fever, thrombocytopenia, and MAHA

51

When might you give cryoprecipitate

For coagulation factor deficiencies involving fibrinogen and factor VIII

52

High leukocyte alkaline phosphatase means what?

Leukemoid reaction

53

What are Dohle bodies?

Light blue (basophilic) peripheral granules in neutrophils seen in a leukemoid reaction

54

What is Hodgkin lymphoma associated with?

EBV

55

Cell markers in reed Sternberg cells

CD15 and CD30

56

Genetics of Burkitt lymphoma

T(8;14) translocation of c-myc and heavy-chain Ig

57

What is commonly involved in diffuse large B cell lymphoma

Waldeyer's ring (oropharyngeal lymphoid tissue) and GI tract

58

Genetics of follicular lymphoma

T(14;18)-translocation of heavy-chain Ig and Bcl-2. Cleaved nuclei on histo

59

Genetics of mantle cell lymphoma

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

60

Cerebriform nuclei

Mycoses fungoides/Sezary syndrome

61

Rouloux formation

Multiple myeloma

62

Neutrophils with bilobed nuclei seen after Chen

Pseudo-Pelger-Huet anomaly

63

Child with lytic bone lesions and skin rash or as recurrent otitis media with a mass involving mastoid bone

Langerhans cell histiocytosis. Calls express S100 (mesodermal origin) and CD1a. Bit beck granules (tennis rackets on EM)

64

T(12;21)

Better prognosis ALL

65

Smudge cells

Small lymphocytic lymphoma/CLL

66

Tx for CML

Imatinib (a small molecule inhibitor of the bcr-abl tyrosine kinase)

67

Mechanism of HIT

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

68

Direct thrombin inhibitors

Argatroban, bivalirudin, dabigatran

69

MOA of cilostazol, dipyridamole

Phosphodiesterase III inhibitor. Increases cAMP in platelets, resulting in inhibition of platelet aggregation; vasodilators

70

MOA of hydroxyurea for cancer

Inhibits ribonucleotide reductase

71

Clinical use for cytarabine

Leukemias (AML), lymphoma

72

Use and toxicity of Busulfan

CML. Also used to ablate patient's bone marrow before bone marrow transplantation. Toxicity: severe myelosuppression