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Weir - Hematology > 3.25.14* Myeloproliferative Disorders > Flashcards

3.25.14* Myeloproliferative Disorders Flashcards

PPT* Lecture Notes* Reading (pp. 192-213)* Pictures

1
Q

hematologic malignancy

A

a. there is too much of stuff-unregulated growth or two little apoptosis
b. maturation arrest-

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2
Q

*Mechanism of action of JAK-2 V617F mutation

A

constitutive activation of Epo receptor leads to

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3
Q

*Diagnostic criteria for P Vera

A

a. JAK-2 V617Fn (95%)

b. Hgb over 18.5 in men and 16.5 in women or other evidence of increased red cell volume

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4
Q

*Diagnostic criteria for ET

A

a. 50-60 y/o
b. high platelets (and WBC)
c. Jak2V617F
d. causes thrombotic occlusions called erythromelalgia.
e. PBS: giant platelets

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5
Q

*Diagnostic criteria for primary myelofibrosis

A

a. hepatosplenomegaly, anemia, leukocytosis, thrombocytosis
b. PBS- nucleated RBC, giant platelets, teardrop RBCS, left shift
c. reticulin or collagen fibrosis
d. treatment: Jak2 inhibitor, BMT

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6
Q

*Diagnostic criteria for chronic myeloid leukemia

A

a. hepatosplenomegaly, over 60
b. PBS: elevated EBC, platelets, basophilia. All stages of granulocyte differentiation (look like HSV).
c. bone marrow: hypercellularity, increased ratio of myeloid cells to erythroid cells, increased megs.

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7
Q

*three causes of secondary polycythemia

A

chronic hypoxia (sleep apnea)
Epo secreting tumors
high affinity hemoglobin

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8
Q

*treatment of P vera

A

a. phlebotomy
b. aspirin (to reduce thrombosis)
c. hydroxyurea (to reduce thrombosis)
d. interferon (reverses increased platelets/WBC and splenomegaly)
e. JAK2 inhibitor

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9
Q

*mutation in mastocytosis

A

c-KIT leading to mast cell activation/proliferation

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10
Q

*mutation in chronic eosinophilic leukemia

A

PDGFRA

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11
Q

*organs damaged in eosinophilia

A 
heart
lung
GI
thromboses
eczema
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12
Q

*causes of secondary eosinophilia

A

drug hypersensitivity, infection, hypoadrenalism, and solid organ neoplasm

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13
Q

*Mutation and mechanism of action of Philadelphia chromosome

A

BCR-ABL in CML causing constitutive tyrosine kinase activaiton

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14
Q
  • Mechanism of action of present best therapy for CML
A

tyrosine kinase inhibitor (imatinib)

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15
Q

*Peripheral blood picture in CML

A

Looks like bone marrow without megakaroyctes because of so many white cells in the blood. Usually just bands and segs, but when you see blasts, myelocyte, promyelocyte in the peripheral blood there is something wrong if you don’t have sepsis.

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16
Q

The MPN category includes

A 
chronic myelogenous leukemia (CML)
polycythemia vera (PV)
essential thrombocythemia (ET)
primary myelofibrosis (PMF)
chronic neutrophilic leukemia (CNL)
chronic eosinophilic leukemia
Systemic mastocytosis (SM)
17
Q 
Which does not belong when explaining myeloproliferative neoplasms?
Myeloid accumulation
Clonal
Stem cell mutations
Full maturation
Dysplastic differentiation
A

(there is a myeloid differentiation, the mutation is all the way back in the stem cells)

18
Q

Symptoms of polycythemia vera

A

itch (due to histamine release)
headaches
fatigue

19
Q

Signs of polycythemia vera

A

plethora
splenomegaly
conjunctival plethora

20
Q

what is the main cause of death in patients with PV

A

thrombosis

acute myeloid leukemia

21
Q

What is seen in PBS of P vera

A

often hypochromic microcytic due to iron deficiency (from GI bleeding)
Elevated RBC, WBC and platelets

22
Q

What is seen in bone marrow of P vera

A

hypercellular

loose clusters of pleomorphic megakaryocyttes

23
Q

What happens in PV, post-polycythemia phase

A

marrow fibrosis

24
Q

polycythemia vera

A

a. panmyelosis that is monoclonal

b. often mild

25
Q

What is the difference between PV and ET

A

red cell mass

both have increased platelets and are Jak2 positive

26
Q

ET therapy

A

a. hydroxyurea (reduced platelet count)
b. anagrelide (decrease platelets)
c. aspirin

27
Q

patient has massive reticulin deposition in bone marrow

A

gonna need a bone marrow transplant

28
Q

what is seen in mastocytosis?

A

a. urticaria pigmentosis- small itchy rash due to mast cell release

29
Q

treatment for mastocytosis

A

a. prophylaxis to block mast cell mediators

b. interferon plus corticosteroids

30
Q

treatment for CML

A

imatinib

allogenic transplants

Weir - Hematology (6 decks)

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