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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
increased and morphologically abnormal mast cells in various tissues
mastocytosis
26
initially asymptomatic non-specific splenomegaly thrombosis or bleeding
CML
27
increased RBC mass: plethora, headache, dizziness, visual disturbances, angina splenomegaly thrombosis or bleeding
PV
28
non-specific symptoms splenomegaly infections, hemmorhage
PMF
29
splenomegaly can cause
early satiety, feelings of fullness, LUQ pain
30
thrombosis of large vessels bleeding due to platelet functional abnormalities erythromelagia
ET
31
urticaria pigmentosa in children
mastocytosis
32
mastocytosis can cause what systemic disease in 10% of adults?
systemic mastocytosis
33
leukocytosis >20,000 per uL **often >100,000
CML
34
elevated RBC, hemoglobin, hematocrit low or normal serum EPO
PV
35
leukoerythroblasts tear-drop shaped RBCs
PMF
36
marked thrombocytosis (>450,000) with abnormal morphology circulating megakaryotic fragments
ET
37
decreased or absent iron
PV
38
markedly hypercellular with granulocyte predominance in bone marrow
CML
39
reticulin and collagenous fibrosis, osteosclerosis in bone marrow
PMF
40
bone marrow positive for metachromatic stains, CD117, tryptase
mastocytosis
41
bone marrow blasts <10%
CML
42
CML immature granulocyte predominance (neutrophils, eosinophils, basophils)
43
CML hypercellular, very little fat
44
PV erythroid predominance
45
PMF myelo and metamyelocytes
46
PMF top dark purple: nucleated RBC
47
PMF tear drop shaped RBCs
48
PMF lots of collagen in marrow
49
ET lots of platelets
50
ET megakaryocytes
51
mastocytosis
52
mastocytosis bottom left: typtase staining bottom right: CD117 staining
53
diagnostic findings associated with significant splenomegaly
expanded red pulp (extramedullary hematopoiesis) fibrosis infarcts
54
spleen megakaryocytes and blasts indicating extramedullary hematopoiesis
55
CML blast crisis
56
once CML has progressed to blast crisis, prognosis is
<1 year
57
70% myeloid
AML
58
30% lymphoid
ALL
59
managed with phlebotomy
PV
60
associated with massive splenomegaly [2]
PMF and CML
61
non-neoplastic proliferations of cells [3]
neutrophillia erythrocytosis thrombocytosis
62
caused by acute phase reactants, iron deficiency, drugs
thrombocytosis
63
appropriate increased EPO is caused by
hypoxia high altitudes, smoker, etc.
64
caused by infections, immune disorders, drugs
neutrophilia
65
Dohle bodies, toxic granules
neutrophilia
66
dohle body
67
toxic granules
68
metamyelocytes and myelocytes are precursors of
neutrophils
69
translocation associated with CML
t(9;22) | causes BCR/ABL translocation
70
translocation associated with CML
t(9;22) | causes BCR/ABL translocation
71
in adult life, hematopoiesis occurs more in the
flat bones
72
hematopoiesis during fetal development occurs in
liver and spleen
73
hematopoiesis occurs in the ...... in first trimester
yolk sac
74
as we age, hematpoietic bone marrow is reduced in
the long bones | occurs more in flat bones
75
as we age, hematpoietic bone marrow is reduced in
the long bones | occurs more in flat bones
76
serotonin and histamine
mast cells
77
major basic protein
eosinophil
78
absolute basophilia and eosinophilia
CML
79
reactive/secondary erythropoiesis would have
increased EPO levels
80
the bone marrow in PV is
hypercellular
81
iron stores in PV are depleted due to
overproduction of RBCs
82
t(8;14)
Burkitt lymphoma
83
(t9;22)
CML
84
t(15;17)
APL
85
required for diagnosis of CML
t(9;22)

IBI Test 3 (20 decks)

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