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Hematology > 99 PV > Flashcards

99 PV Flashcards

1
Q

What are the 8 WHO classification of chronic myeloproliferative neoplasm

A
  1. CML, bcr-abl positive
  2. Chronic neutrophilic leukemia
  3. Chronic eosininophilic leukemia, not otherwise specified
  4. Polycythemia vera
  5. Primary myelofibrosis
  6. Essential thrombocytosis
  7. Mastocytosis
  8. Myeloproliferative neoplasms, unclassified
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2
Q

associated with balanced translocation between chromosomes 9 and 22`

A

CML

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3
Q

associated with a t(15:19)

A

Chronic neutrophilic leukemia

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4
Q

tyrosine kinase essential for the function of erythropoeitin and thrombopoietin

A

JAK2

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5
Q

The mutation seen in PV, PMF, and ET

A

mutation in V617F

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6
Q

True or false. In CML, CNL, there is a high rate of leukemic transformation due to its mutation effects on the granulocyte colony stimulating factor receptor

A

True.

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7
Q

True or false. In PV, ET, PMF there is rare transformation to leukemia unless there is exposure to mutagenic drugs.

A

True.

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8
Q

Most common of the chronic myeloproliferative neoplasm

A

Polycythemia vera

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9
Q

Clonal disorder involving multipotent hematopoetic progenitor cell in which phenotypically normal red cells, granulocytes, and platelets accumulate in the absence of a recognizable physiologic stimulus

A

Polycythemia vera

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10
Q

True or false. No cosistent cytogenic abnormality is associated with PV unlike CML

A

True.

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11
Q

central role in the pathogenesis of PV

A

mutation in the autoinhibitory pseudokinase domain of tyrosine kinase JAK2 that replaces valine with phenylalanine (V617F)

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12
Q

member of an evolutionary well conserved non receptor tyrosine kinase family and serves as cognate tyrosine kinase for the erythropoetin or thrombopoeitin receptors

A

JAK2

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13
Q

True or false. JAK2V617F is the basis for many of the phenotypic and biochemical characteristics of PV such as elevation of leukocyte alkaline phosphatase (LAP) score

A

True.

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14
Q

How is PV usually first recognized

A

incidental discovery of high hemoglobin or hematocrit

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15
Q

With the except of this manifestation, no symptoms distinguish PV from other causes of erythrocytosis

A

aquagenic pruritus

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16
Q

initial presenting manifestations of PV

A

isolated thrombocytosis, leukocytosis or splenomegaly

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17
Q

True or false. Systolic hypertension is also a feature of red cell mass elevation

A

True.

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18
Q

True or false. Uncontrolled erythrocytosis causes hyperviscosity leading to neurologic symptoms such as vertigo, tinnitus, headache, visual disturbances and TIAs.

A

True.

19
Q

most commonly affected vessels in PV

A

cerebral, cardiac, mesenteric vessels

20
Q

True or false. Venous or arterial thrombosis may be the presenting manifestations of PV

A

True.

21
Q

complications of PV manifested as erythema, burning, and pain in the extremities

A

erythromelalgia

22
Q

what is mechanism of erythromelalgia

A

due to increased platelet stickiness

23
Q

What is unique in PV in contrast to other causes of erythrocytosis

A

expansion of plasma volume

24
Q

What is the hemoglobin or hematocrit level to say it is undeniably PV?

A

hgb more than 20 g/dl or hematocrit more than 60%

25
Q

3 situations where there is microscopic erythrocytosis

A

beta thalassemia trait, hypoxic erythrocytosis, and polycythemia vera

26
Q

other names of stress erythrocytosis

A

spurious erythrocytosis or Gaisbocks syndrome

27
Q

how to differential PV from beta thalassemia trait

A

in beta thalassemia, the RDW is normal while it is elevated in PV

28
Q

True or false. JAK2 assay has superseded other tests for establishing the diagnosis of PV

A

True.

29
Q

True or false. Bone marrow aspirate and biopsy provide no specific diagnostic information because these may be normal or indistinguishable from ET or PMF

A

True.

30
Q

True or false. The incidence of H Pylori infection in PV is increased

A

True.

31
Q

What is the pathophysiology of pruritus in PV

A

consequence of mast cell activation by JAK2V617F

32
Q

True or false. normal hematocrit or hemoglobin with massive splenomegaly should be considered indicative of elevated red cell mass until proven otherwise

A

True.

33
Q

True or false. In patients with splenomegaly, true extent of red cell mass elevation is masked

A

True.

34
Q

most significant complication of PV

A

thrombosis

35
Q

what is the target Hgb and Hct levels in PV to avoid thrombotic complications

A

Men: Hgb less than 14 g/dL and Hct less than 45%
Women: Hgb less han 12 g/dl and Hct less than 42%

36
Q

How many phlebotomy or when is it required

A

Once patient is in iron deficient state, phlebotomy may be done at 3 month interval

37
Q

serum uric acid value when allopurinol is given

A

serum uric acid of 10 mg/dL and above

38
Q

True or false. Due to an elevated platelet count, PV may manifest with acquired vWF deficiency

A

True.

39
Q

phosphodiesterase inhibitor that can be given to reduce platelet count with lack of marrow toxicity and protective against venous thrombosis

A

anagrelide

40
Q

True or false. Allogeneic bone marrow transplant is promising in PV

A

False. No role

41
Q

tyrosine kinase inhibitor for PV patients unresponsive to cytotoxic agent

A

ruxolitinib

42
Q

Least common MPN

A

primary myelofibrosis

43
Q

clonal disorder characterized by multipotent hematopoietic progenitor cell of unknown etiology characterized by marrow fibrosis, extramedullary hematopoeisis and splenomegaly

A

Primary myelofibrosis

Hematology (11 decks)

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