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IBI Test 3 > B3-040 Blood Cell Disorders > Flashcards

B3-040 Blood Cell Disorders Flashcards

1
Q

if there’s blasts in the blood sample, what test should be done next?

A

flow cytometry

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2
Q

neoplasms of hematopoietic stem cells with proliferation of one or more of the myeloid lineages with accumulation of maturing or mature cells

A

myeloproliferative neoplasms

**proliferation with maturation

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3
Q

myeloproliferative neoplasms [5]

A

chronic myeloid leukemia (CML)
polycythemia vera (PV)
primary myelofibrosis (PMF)
essential thrombocytosis (ET)
mastocytosis

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4
Q

myeloproliferative neoplasms are caused by mutated

A

tyrosine kinases

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5
Q

t(9;22)

A

CML

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6
Q

P210 fusion protein from BCR/ABL-1 fusion

A

CML

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7
Q

most myeloproliferative neoplasms are caused by a ________ mutation in ________

A

point; psuedokinase domain of JAK2
on chr 9

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8
Q

the tyrosine kinase mutation causes

A

loss of autoinhibition; constitutive phosphorylation of STAT5

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9
Q

JAK2 inhibitor [drug]

A

ruxolitinib

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10
Q

P210 fusion protein inhibitor for CML [drug]

A

imatinib mesylate (Gleevec)

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11
Q

present in all cases of PV and up to 50% of PMF and ET cases

A

JAK2 point mutation

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12
Q

mutated chaperone protein causing 25-35% of PMF and ET

A

calreticulin

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13
Q

mutated protein causing mastocytosis

A

KIT

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14
Q

thromboproietic receptor mutation causing a small amount of PMF and ET

A

MPL

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15
Q

neoplasm of pluripotential hematopoietic stem cell

A

CML

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16
Q

marked granulocytosis including all stages of maturation

A

CML

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17
Q

BCR-ABL rearrangement

A

CML

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18
Q

neoplasm of multipotential hematopoietic stem cell, predominantly effects erythroid lineage

A

PV

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19
Q

markedly increased total body erythrocyte mass

A

PV

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20
Q

neoplasm of multipotential hematopoietic stem cell with reactive fibrosis

A

PMF

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21
Q

pro-fibrotic growth factors (PDGF, TGF-b) from megakaryoctes

A

PMF

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22
Q

neoplasms of multipotential hematopoietic stem cell with uncontrolled proliferation of megakaryocytes

A

ET

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23
Q

marked increase in platelets and abnormal platelet function

A

ET

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24
Q

neoplasm of mast cells

A

mastocytosis

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25
Q

increased and morphologically abnormal mast cells in various tissues

A

mastocytosis

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26
Q

initially asymptomatic
non-specific
splenomegaly
thrombosis or bleeding

A

CML

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27
Q

increased RBC mass: plethora, headache, dizziness, visual disturbances, angina
splenomegaly
thrombosis or bleeding

A

PV

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28
Q

non-specific symptoms
splenomegaly
infections, hemmorhage

A

PMF

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29
Q

splenomegaly can cause

A

early satiety, feelings of fullness, LUQ pain

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30
Q

thrombosis of large vessels
bleeding due to platelet functional abnormalities
erythromelagia

A

ET

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31
Q

urticaria pigmentosa in children

A

mastocytosis

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32
Q

mastocytosis can cause what systemic disease in 10% of adults?

A

systemic mastocytosis

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33
Q

leukocytosis >20,000 per uL

**often >100,000

A

CML

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34
Q

elevated RBC, hemoglobin, hematocrit
low or normal serum EPO

A

PV

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35
Q

leukoerythroblasts
tear-drop shaped RBCs

A

PMF

36
Q

marked thrombocytosis (>450,000) with abnormal morphology
circulating megakaryotic fragments

A

ET

37
Q

decreased or absent iron

A

PV

38
Q

markedly hypercellular with granulocyte predominance in bone marrow

A

CML

39
Q

reticulin and collagenous fibrosis, osteosclerosis in bone marrow

A

PMF

40
Q

bone marrow positive for metachromatic stains, CD117, tryptase

A

mastocytosis

41
Q

bone marrow blasts <10%

A

CML

42
Q
A

CML

immature granulocyte predominance (neutrophils, eosinophils, basophils)

43
Q
A

CML

hypercellular, very little fat

44
Q
A

PV

erythroid predominance

45
Q
A

PMF

myelo and metamyelocytes

46
Q
A

PMF

top dark purple: nucleated RBC

47
Q
A

PMF

tear drop shaped RBCs

48
Q
A

PMF

lots of collagen in marrow

49
Q
A

ET

lots of platelets

50
Q
A

ET

megakaryocytes

51
Q
A

mastocytosis

52
Q
A

mastocytosis

bottom left: typtase staining
bottom right: CD117 staining

53
Q

diagnostic findings associated with significant splenomegaly

A

expanded red pulp (extramedullary hematopoiesis)
fibrosis
infarcts

54
Q
A

spleen

megakaryocytes and blasts indicating extramedullary hematopoiesis

55
Q
A

CML

blast crisis

56
Q

once CML has progressed to blast crisis, prognosis is

A

<1 year

57
Q

70% myeloid

A

AML

58
Q

30% lymphoid

A

ALL

59
Q

managed with phlebotomy

A

PV

60
Q

associated with massive splenomegaly [2]

A

PMF and CML

61
Q

non-neoplastic proliferations of cells [3]

A

neutrophillia
erythrocytosis
thrombocytosis

62
Q

caused by acute phase reactants, iron deficiency, drugs

A

thrombocytosis

63
Q

appropriate increased EPO is caused by

A

hypoxia

high altitudes, smoker, etc.

64
Q

caused by infections, immune disorders, drugs

A

neutrophilia

65
Q

Dohle bodies, toxic granules

A

neutrophilia

66
Q
A

dohle body

67
Q
A

toxic granules

68
Q

metamyelocytes and myelocytes are precursors of

A

neutrophils

69
Q

translocation associated with CML

A

t(9;22)

causes BCR/ABL translocation

70
Q

translocation associated with CML

A

t(9;22)

causes BCR/ABL translocation

71
Q

in adult life, hematopoiesis occurs more in the

A

flat bones

72
Q

hematopoiesis during fetal development occurs in

A

liver and spleen

73
Q

hematopoiesis occurs in the …… in first trimester

A

yolk sac

74
Q

as we age, hematpoietic bone marrow is reduced in

A

the long bones

occurs more in flat bones

75
Q

as we age, hematpoietic bone marrow is reduced in

A

the long bones

occurs more in flat bones

76
Q

serotonin and histamine

A

mast cells

77
Q

major basic protein

A

eosinophil

78
Q

absolute basophilia and eosinophilia

A

CML

79
Q

reactive/secondary erythropoiesis would have

A

increased EPO levels

80
Q

the bone marrow in PV is

A

hypercellular

81
Q

iron stores in PV are depleted due to

A

overproduction of RBCs

82
Q

t(8;14)

A

Burkitt lymphoma

83
Q

(t9;22)

A

CML

84
Q

t(15;17)

A

APL

85
Q

required for diagnosis of CML

A

t(9;22)

IBI Test 3 (20 decks)

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