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IBI Test 3 > B3.040 - Blood Cell Dyscrasias > Flashcards

B3.040 - Blood Cell Dyscrasias Flashcards

1
Q

Blood Diagnostic findings of ET

A

marked thrombocytosis with abnormal morphology; circulating megakryocytic fragments

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2
Q

mastocytosis bone marrow findings

A

aggregates of mast cells positive for metachromatic stains

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3
Q

what mutation is associated with myeloproliferative neoplasms

A

mutuation constituitively activated tyrosine kinase

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4
Q

CML is a neoplasm of what

A

pluripotential hematopoietic stem cell

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5
Q

imatinib aka

A

gleevec

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6
Q

clinical features of PMF

A

non specific symptoms - fatigue, malaise, weight loss splenomegaly, often massive infections, hemorrhage

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7
Q

Blood Diagnostic findings of CML

A

leukocytosis >20,000 all stages of maturation basophilia

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8
Q

PMF is neoplasm of what

A

multipotential hematopoietic stem cell with reactive fibrosis

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9
Q

Myeloid neoplasms

A

myeloproliferative neoplasms acute myeloid leukemia myelodysplastic syndromes

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10
Q

clinical features of CML

A

initially asymptomatic fatigue, malaise, fever, weight loss splenomegaly thrombosis or bleeding

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11
Q

Blood Diagnostic findings of PMF

A

leukoerythroblastosis Prominent anisopoikilocytosis with tear drop shaped RBCs

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12
Q

what mutation is associated with PV and up to 50% of cases of PMF/ET

A

JAK2

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13
Q

PMF has what factors

A

profibrotic growth factors (PDGF, TGFbeta) from megakryocytes

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14
Q

Blood diagnostic findings of PV

A

elevated RBC Low or normal serum erythropoietin

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15
Q

what is a diagnostic hallmark of CML

A

BCR/ABL fusion gene

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16
Q

PV features markedly increased what

A

total body erythrocyte mass

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17
Q

what is distinctive of ET

A

markedly increased platelets and abnormal platelet function

18
Q

What is ET a neoplasm of

A

multipotential hematopoietic stem cell with uncontrolled proliferation of megakaryocytes

19
Q

what does the JAK2 mutation do

A

promotes cytokine independent growth as well as hypersensitivity to growth fators

20
Q

PV clinical features

A

increased RBC mass - plethora, headache, dizziness, visual disturbances, angina, intermittent claudation splenomegaly, thrombosis or bleeding

21
Q

what neoplasm is associated with the granulocytic pathway

A

CML

22
Q

mastocytosis is noplasm of

A

mast cells

23
Q

is JAK2 mutation specific to a disease?

A

no

24
Q

what drug targets the JAK2 mutation

A

ruxolitinib

25
Q

PV is a neoplasm of what

A

multipotential hematopoietic stem cells, predominat effects on erythroid lineage

26
Q

what drugs can target TK

A

imatinib

27
Q

why doesn’t our immune system attack imatinib

A

its intracellular

28
Q

Clinical features of mastocytosis

A

urticaria pigmentosa - localized cutaneous disease symptomatic mastocytosis - multiorgan involvement

29
Q

what neoplasm is associated with the platelet pathway

A

ET

30
Q

what disease is associated with the BCR-ABL rearrangement

A

CML

31
Q

myeloproliferateive neoplamsms are proliferation…

A

with maturation

32
Q

bone marrow diagnositc findings PMF

A

hypercellular - mostly granulocytic and megakaryocytci reticulin and collagenous fibrosis and osteosclerosis

33
Q

ET bone marrow findings

A

similar to PMF but no fibrosis

34
Q

bone marrow findings for PV

A

decreased or absent iron marked hypercellular with erythroid predominace

35
Q

why are side effects of JAK2 worse than imatinib?

A

JAK2 is used widely in body so blocking it stops other things too

36
Q

lymphoid neoplasms

A

precursor B/T lymphoblastic leukemia/lymphoma Mature B cell neoplasms Mature T cell neoplasms Hodgkin lymphoma

37
Q

bone marrow CML findings

A

markedly hypercellular with granulocyte predominanc Blasts under 10% Mild fibrosis

38
Q

what disease is associated with parked granulocytosis including all stages of maturatin

A

CML

39
Q

clinical feature of ET

A

often asymptomatic thrombosis of large vessels - lower extremities, heart, intestines, kidney bleeding due to platelet functional abnromailites erythromelagia

40
Q

What are examples of myeloproliferative neoplasms

A

CML PV PMF ET mastocytosis

41
Q

what is the philadelphia chromosome and what is it diagnostic for

A

t(9;22)(q34;q11) BCR/ABL fusion gene diagnostic for CML

42
Q

what neoplasm is associated with the erythroid pathway

A

PV

IBI Test 3 (16 decks)

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