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

B3.040 Blood Cell Disorders Flashcards

1
Q

how do you get an absolute count of a specific WBC?

A

differential count (%) * total WBC count

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2
Q

what are myeloproliferative neoplasms?

A

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

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3
Q

5 principle myeloproliferative disorders

A 
chronic myeloid leukemia (CML)
polycythemia vera (PV)
primary myelofibrosis (PMF)
essential thrombocytosis (ET)
mastocytosis
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4
Q

what is the underlying cause of myeloproliferative neoplasms?

A

mutated, constitutively activated tyrosine kinase

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5
Q

what is a diagnostic hallmark of CML?

A

BCR/ABL-1 fusion
t(9;22) promoter drives production of TK from the ABL region, making it constitutively expressed
Philadelphia chromosome produces novel protein product & lots of it

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6
Q

what is the product of BCR/ABL-1 fusion in CML?

A

P210 fusion protein

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7
Q

what is the significance of the P210 fusion protein?

A

has tyrosine kinase activity

since the mutation is intracellular, it evades the immune system

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8
Q

how is P210 fusion protein inhibited?

A

small molecule inhibitors
enters cell, and competitively binds ATP binding site of the kinase
doesn’t kill cell, just blocks its function

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9
Q

what is the result of a JAK2 mutation?

A

point mutation in pseudokinase domain
pseudokinase domain is supposed to inhibit JAK2, but can’t when mutated
loss of autoinhibition
increased phosphorylation of downstream targets

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10
Q

what effect does increased phosphorylation of downstream targets like STAT5 have on the cell?

A

promotes cytokine independent growth

hypersensitivity to growth factors (EPO and IL3)

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11
Q

what myeloproliferative disorders are/are not associated with JAK2 mutations?

A

almost all PV cases
50% of PMF and ET cases
NOT PRESENT in CML or lymphoproliferative disorders

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12
Q

what other genetic abnormalities are associated with specific myeloproliferative disorders?

A

calreticulin - 25-35% of PMF and ET
MPL (TPO receptor) - in 1-10% of PMF and ET
KIT - in mastocytosis

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13
Q

general features of CML

A

neoplasm of pluripotential hematopoietic stem cell (can be myeloid or lymphoid)
marked granulocytosis including all stages of maturation
BCR-ABL rearrangement

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14
Q

general features of PV

A

neoplasm of multipotential hematopoietic stem cell, predominant effects on erythroid lineage
markedly increased total body erythrocyte mass

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15
Q

general features of PMF

A

neoplasm of multipotential hematopoietic stem cell with reactive fibrosis
probiotic growth factors (PDGF, TBF-B) from megakaryocytes

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16
Q

general features of ET

A

neoplasm of multipotential hematopoietic stem cell with uncontrolled proliferation of megakaryocytes without fibrosis growth factors
markedly increased platelets and abnormal platelet function

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17
Q

general features of mastocytosis

A

neoplasm of mast cells

increased and morphologically abnormal mast cells in various tissues

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18
Q

clinical features of all myeloproliferative disorders

A

slow growing/ slowly progressing
diagnosed after several months to years
incidental discovery common

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19
Q

clinical features of CML

A

initially asymptomatic
non spec symptoms- fatigue, malaise, fever, weight loss
splenomegaly - often massive leading to early satiety, LUQ discomfort
thrombosis or bleeding (due to widespread consequences on cell lines)

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20
Q

clinical features of PV

A

increased RBC mass - plethora, headache, dizziness, visual disturbances, angina, intermittent claudication
splenomegaly
thrombosis or bleeding
sludging and resulting local hypoxia/ischemic events

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21
Q

clinical features of PMF

A

non spec- fatigue, malaise, weight loss
splenomegaly - often massive leading to early satiety, LUQ discomfort
infections
hemorrhage

22
Q

clinical features of ET

A

often asymptomatic
thrombosis of large vessels- lower extremities, heart, intestines, kidney
bleeding due to platelet functional abnormalities
erythromelalgia (intense burning and redness of extremities)

23
Q

clinical features of mastocytosis

A

urticaria pigmentosa - localized cutaneous disease (50%), usually in kids
systemic mastocytosis - multi organ involvement (10%), in adults

24
Q

blood diagnostic findings of CML

A

leukocytosis >20,000, often >100,000
all stages of maturation: myelocytes and neutrophils predominant
basophilia, eosinophilia, mild anemia, thrombocytosis

25
Q

blood diagnostic findings of PV

A

elevated RBS, hemoglobin, hematocrit
normochromic, normocytic RBCs (can be hypo/micro when iron def manifests bc iron is used up from production of unnecessary RBCs)
mild to moderate leukocytosis, thrombocytosis, eosinophilia and basophilia
low or normal serum EPO (suppressed)

26
Q

blood diagnostic findings of PMF

A

leukoerythroblastosis

prominent anisopoikilocytosis w tear drop shaped RBCs

27
Q

blood diagnostic findings for ET

A

marked thrombocytosis (>450,000) with abnormal morphology; circulating megakaryocytic fragments
mild anemia
mild to moderate leukocytosis

28
Q

bone marrow diagnostic findings for CML

A

markedly hypercellular with granulocyte predominance
blasts <10%
mild fibrosis

29
Q

bone marrow diagnostic findings for PV

A

markedly hypercellular with erythrocyte predominance
increased and clustered megakaryocytes
decreased or absent iron
mild to moderate fibrosis

30
Q

bone marrow diagnostic findings for PMF

A

hypercellular - mostly granulocytic and megakaryocytic
increased, clustered, atypical megakaryocytes
reticulin and collagenous fibrosis and osteosclerosis

31
Q

bone marrow diagnostic findings for ET

A

variably hypercellular with megakaryocytic predominance
sheets and clusters of atypical megakaryocytes
mild or no fibrosis

32
Q

bone marrow diagnostic findings for mastocytosis

A

aggregates of mast cells - positive for metachromatic stains (toluidine blue), CD 117, tryptase

33
Q

histo features of CML

A

different stages of neutrophils
heavily weighted towards granulocytes in blood and marrow
lots of cellularity in marrow

34
Q

histo features of PV

A

round nuclei aka nucleated RBCs in marrow

35
Q

histo features of PMF

A

tear drop cells
precursors of erythroid and myeloid cells in blood (marrow letting it out early due to fibrosis distortions)
in marrow you can stain for collagen to see fibrosis

36
Q

histo features of ET

A

lots of platelets in blood

lots of megakaryocytes in marrow

37
Q

histo features of mastocytosis

A

lots of cells in the dermis
ovoid nuclei and granular cytoplasm
stains for CD117

38
Q

what are some diagnostic findings from the spleen?

A 
significant splenomegaly
-expanded red pulp with extramedullary hematopoiesis
-fibrosis
-infarcts
(due to thrombi formation)
39
Q

course and prognosis of chronic phase CML

A

chronic phase - 2-8 years; prolonged with TKI therapy

40
Q

course and prognosis of accelerated phase CML

A

accelerated phase - blasts 10-19%, increased basophils, persistent thrombocytopenia or thrombocytosis, increased splenomegaly and WBC, karyotypic evolution

41
Q

course and prognosis of blast crisis CML

A

blast crisis- survival <1 y

-blasts >20% (acute leukemia)

42
Q

course and prognosis of PV

A

proliferative phase- erythroid proliferation
-median survival 10 y, manage w phlebotomy
spent phase - stable or decreased erythroid mass
post-polycythemic myelofibrosis- 15-20%
AML - 2%

43
Q

course and prognosis of PMF

A

median survival - 3-5 years

AML - 5-20%

44
Q

course and prognosis of ET

A

median survival - 10 years

AML - rare

45
Q

what is the primary difference between myeloproliferative neoplasms and non-neoplastic proliferations of cells?

A

non-neoplastic lack genetic abnormalities (non-clonal)
far more common
can differentiate with BCR/ABL or JAK2 testing

46
Q

what are some non-neoplastic causes of neutrophilia

A

infections (primarily) bacterial, immunological inflammatory disorders, neoplasia (paraneoplastic syndrome), drugs

47
Q

what are some associated features of neutrophilia?

A

toxic granulation

Dohle bodies

48
Q

what are some non-neoplastic causes of erythrocytosis?

A

relative due to hemoconcentration
increased EPO due to hypoxia (altitude, COPD, heart disease)
increased EPO from tumors (paraneoplastic syndrome with other tumors)

49
Q

what are some non-neoplastic causes of thrombocytosis?

A

acute phase reaction
iron def
drugs

50
Q

what is a Dohle body

A

blue areas of basophilia inside neutrophils

IBI Test 3 (19 decks)

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