WBC Part 7 Flashcards

1
Q

Myelodysplastic Syndromes (MDS) are abnormalities in what that result in what

A

clonal multipotent stem cell abnormality resulting in ineffective hematopoiesis with dysplasia

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2
Q

what type of patient would one expect to see myelodysplastic syndrome in

A

older patient with refractory pancytopenia or macrocyric anemia
someone who had radiation or chemo 2-7 years prior

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3
Q

what does one see in the peripheral blood of a patient with mylelodysplastic syndromes (MDS)

A

pancytopenia with macrocytic anemia

dysplastic neutrophils

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4
Q

what does one see in the bone marrow of a patient with mylelodysplastic syndromes (MDS)

A

packed marrow
trilineage hyperplasia with dysplasia in at least one line
increased storage iron
blasts less than 20%

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5
Q

ineffective hematopoiesis with dysplasia is seen in what

A

myelodysplastic syndromes

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6
Q

mylelodysplastic syndromes (MDS) prognosis depends on what

A

% blasts
amount of dysplasia
complex cytogenetics
therapy related

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7
Q

what is the most common cytogenetic change seen in mylelodysplastic syndromes (MDS)

A

monosomy (5 or 7)
del5q, del7q, del20q
trisomy 8
chromosomal abnormalities seen in about 50% of MDS

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8
Q

Myelodisplastic syndromes are a group of disorders, what are the high and low grade disorders

A
Refractory Anemia (low grade)
Refractory Anemia with Excess Blasts (high grade)
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9
Q

low grade MDS is diagnosed how

A

exclusion

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10
Q

MDS can progress to what

A

AML

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11
Q

how are myelodisplastic syndromes treated

A

young patient- allogenic BM treatment

older patient- supportive therapy

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12
Q

Chronic Myeloproliferative Diseases (CMPD) is characterized by what kind of hematopoiesis

A

effective hematopoiesis just too much of it

can give rise to extramedulary hematopoiesis

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13
Q

Chronic Myeloproliferative Diseases (CMPD) have what associated cellularity in the BM

A

hypercellularity with associated increased peripheral sounds

no dysplasia

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14
Q

What conditions are Chronic Myeloproliferative Diseases (CMPD)

A
Chronic Myeloid Leukemia (CML)
Polycythemia Vera (PV)
Essential Thrombocytopenia (ET)
Primary Myelofibrosis
Chronic Eosinophilic Leukemia/Hypereosinophilic Syndrome
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15
Q

Chronic Myeloid Leukemia (CML) is what mutation and in what type of cell

A

pluripotent stem cell

t(9;22) BCR-ABL fusion gene

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16
Q

how can one ID the BRC-ABL gene

A

FISH or PCR

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17
Q

what is seen in the peripheral blood of CML patient

A

leukocytosis and neutrophilic with left shift
eosinophilia and basophilia
thrombocytosis in 50%

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18
Q

what is seen in the BM of a CML patient

A

packed marrow with prominent granulocytic hyperplasia

many small megakaryocytes

19
Q

what happens to CML if not treated

A

slow progression to AML/ALL

20
Q

what drug inhibits BCR-ABL kinase activity

A

Gleevac

21
Q

in order to diagnose polycythemia vera what must be ruled out

A

secondary polycythemia

22
Q

polycythemia vera is an issue with what cells and due to what mutation in 95% of cases

A

multipotent stem cell

JAK-2 mutation

23
Q

what is seen in the peripheral blood of a polycythemia vera patient

A

decreased serum erythropoietin
erythrocytosis, leukocytosis, thrombocytosis
basophilia

24
Q

what is seen in the bone marrow of polycythemia vera patient

A

hyper cellular with trilineage
no dysplasia
minimal reticulin fibrosis and EMH (late stage can get it)

25
Q

what are the clinical feature related to polycythemia vera

A

late middle age
symptoms related to vascular consequence of increased RBC mass
abnormal blood flow on venous side
headache, dizziness, paresthesia

26
Q

25% Polycythemia vera patients present with what

A

bleeding and thrombosis
(DVT, MI, hepatic, portal and mesenteric veins
ulcers and hemorrhage in GI)

27
Q

how does one treat polycythemia vera

A

phlebotomy to keep hematocrit below 45%

28
Q

erythrocytosis with decreased serum erythropeosis is seen in what

A

polycythemia vera

29
Q

essential thrombocytopenia is diagnosed how

A

diagnosis of exclusion (rule out reactive thrombocytosis and other CMPD)

30
Q

essential thrombocytopenia is characterized by what

A

platelet count of over 600k with abnormal large platelets

31
Q

what mutation is seen in about 50% of essential thrombocytopenia

A

JAK-2

32
Q

what are the clinical features of essential thrombocytopenia

A

asymptomatic

bleeding and thrombosis (from dysfunctional and numerous platelets)

33
Q

what is seen in the BM of essential thrombocytopenia

A

hyper cellular with increased and huge megakaryocytes

mild increased reticulin fibrosis

34
Q

primary myelofibrosis leads to an eventual increase in what resulting in what

A

increased reticule and collagen fibrosis resulting in spent marrow and extra medullary hematopoiesis (ineffective so cytopenias persist)

35
Q

in Primary Myelofibrosis what is released and by what cells

A

neoplastic megakaryocytes release PDGF and TGF-beta (fibroblast mitogens)

36
Q

50% of primary myelofibrosis is due to what mutation

A

JAK-2

37
Q

what are the stages of primary myelofibrosis

A

pre-fribrotic and fibrotic

38
Q

what is the difference between the pre-fibrotic and fibrotic stages of primary myelofibrosis

A

pre-fibrotic is hypercellular in marrow but fibrotic is hypocelular with fibrosis replacing cells
(atypical clusters of megakaryocytic seen both)

39
Q

what are the clinical features of primary myelofibrosis

A

over 60 w/ fatigue w/ progressive anemia, early satiety with splenomegaly and symptoms similar to PV and ET

40
Q

what is seen in chronic eosinophilic leukemia/hypereosinophilic syndrome

A

leukocytosis and absolute eosinophilia

41
Q

eosinophilic leukemia/hypereosinophilic syndrome is due to what

A

fusion of PDGFRalpha and FIP1L1 at 4q12

42
Q

patients with eosinophilic leukemia/hypereosinophilic syndrome respond to what

A

Gleevac

43
Q

extramedullary hematopoiesis and left upper quadrant pain/fullness are clinical features of what

A

CML