B3.040 - Blood Cell Dyscrasias

Question 1

Blood Diagnostic findings of ET

Answer 1

marked thrombocytosis with abnormal morphology; circulating megakryocytic fragments

Question 2

mastocytosis bone marrow findings

Answer 2

aggregates of mast cells positive for metachromatic stains

Question 3

what mutation is associated with myeloproliferative neoplasms

Answer 3

mutuation constituitively activated tyrosine kinase

Question 4

CML is a neoplasm of what

Answer 4

pluripotential hematopoietic stem cell

Question 5

imatinib aka

Answer 5

gleevec

Question 6

clinical features of PMF

Answer 6

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

Question 7

Blood Diagnostic findings of CML

Answer 7

leukocytosis >20,000 all stages of maturation basophilia

Question 8

PMF is neoplasm of what

Answer 8

multipotential hematopoietic stem cell with reactive fibrosis

Question 9

Myeloid neoplasms

Answer 9

myeloproliferative neoplasms acute myeloid leukemia myelodysplastic syndromes

Question 10

clinical features of CML

Answer 10

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

Question 11

Blood Diagnostic findings of PMF

Answer 11

leukoerythroblastosis Prominent anisopoikilocytosis with tear drop shaped RBCs

Question 12

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

Answer 12

JAK2

Question 13

PMF has what factors

Answer 13

profibrotic growth factors (PDGF, TGFbeta) from megakryocytes

Question 14

Blood diagnostic findings of PV

Answer 14

elevated RBC Low or normal serum erythropoietin

Question 15

what is a diagnostic hallmark of CML

Answer 15

BCR/ABL fusion gene

Question 16

PV features markedly increased what

Answer 16

total body erythrocyte mass

Question 17

what is distinctive of ET

Answer 17

markedly increased platelets and abnormal platelet function

Question 18

What is ET a neoplasm of

Answer 18

multipotential hematopoietic stem cell with uncontrolled proliferation of megakaryocytes

Question 19

what does the JAK2 mutation do

Answer 19

promotes cytokine independent growth as well as hypersensitivity to growth fators

Question 20

PV clinical features

Answer 20

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

Question 21

what neoplasm is associated with the granulocytic pathway

Answer 21

CML

Question 22

mastocytosis is noplasm of

Answer 22

mast cells

Question 23

is JAK2 mutation specific to a disease?

Answer 23

no

Question 24

what drug targets the JAK2 mutation

Answer 24

ruxolitinib

Question 25

PV is a neoplasm of what

Answer 25

multipotential hematopoietic stem cells, predominat effects on erythroid lineage

Question 26

what drugs can target TK

Answer 26

imatinib

Question 27

why doesn't our immune system attack imatinib

Answer 27

its intracellular

Question 28

Clinical features of mastocytosis

Answer 28

urticaria pigmentosa - localized cutaneous disease symptomatic mastocytosis - multiorgan involvement

Question 29

what neoplasm is associated with the platelet pathway

Answer 29

ET

Question 30

what disease is associated with the BCR-ABL rearrangement

Answer 30

CML

Question 31

myeloproliferateive neoplamsms are proliferation...

Answer 31

with maturation

Question 32

bone marrow diagnositc findings PMF

Answer 32

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

Question 33

ET bone marrow findings

Answer 33

similar to PMF but no fibrosis

Question 34

bone marrow findings for PV

Answer 34

decreased or absent iron marked hypercellular with erythroid predominace

Question 35

why are side effects of JAK2 worse than imatinib?

Answer 35

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

Question 36

lymphoid neoplasms

Answer 36

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

Question 37

bone marrow CML findings

Answer 37

markedly hypercellular with granulocyte predominanc Blasts under 10% Mild fibrosis

Question 38

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

Answer 38

CML

Question 39

clinical feature of ET

Answer 39

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

Question 40

What are examples of myeloproliferative neoplasms

Answer 40

CML PV PMF ET mastocytosis

Question 41

what is the philadelphia chromosome and what is it diagnostic for

Answer 41

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

Question 42

what neoplasm is associated with the erythroid pathway

Answer 42

PV