99 PV

Question 1

What are the 8 WHO classification of chronic myeloproliferative neoplasm

Answer 1

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

Question 2

associated with balanced translocation between chromosomes 9 and 22`

Answer 2

CML

Question 3

associated with a t(15:19)

Answer 3

Chronic neutrophilic leukemia

Question 4

tyrosine kinase essential for the function of erythropoeitin and thrombopoietin

Answer 4

JAK2

Question 5

The mutation seen in PV, PMF, and ET

Answer 5

mutation in V617F

Question 6

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

Answer 6

True.

Question 7

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

Answer 7

True.

Question 8

Most common of the chronic myeloproliferative neoplasm

Answer 8

Polycythemia vera

Question 9

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

Answer 9

Polycythemia vera

Question 10

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

Answer 10

True.

Question 11

central role in the pathogenesis of PV

Answer 11

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

Question 12

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

Answer 12

JAK2

Question 13

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

Answer 13

True.

Question 14

How is PV usually first recognized

Answer 14

incidental discovery of high hemoglobin or hematocrit

Question 15

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

Answer 15

aquagenic pruritus

Question 16

initial presenting manifestations of PV

Answer 16

isolated thrombocytosis, leukocytosis or splenomegaly

Question 17

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

Answer 17

True.

Question 18

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

Answer 18

True.

Question 19

most commonly affected vessels in PV

Answer 19

cerebral, cardiac, mesenteric vessels

Question 20

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

Answer 20

True.

Question 21

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

Answer 21

erythromelalgia

Question 22

what is mechanism of erythromelalgia

Answer 22

due to increased platelet stickiness

Question 23

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

Answer 23

expansion of plasma volume

Question 24

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

Answer 24

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

Question 25

3 situations where there is microscopic erythrocytosis

Answer 25

beta thalassemia trait, hypoxic erythrocytosis, and polycythemia vera

Question 26

other names of stress erythrocytosis

Answer 26

spurious erythrocytosis or Gaisbocks syndrome

Question 27

how to differential PV from beta thalassemia trait

Answer 27

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

Question 28

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

Answer 28

True.

Question 29

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

Answer 29

True.

Question 30

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

Answer 30

True.

Question 31

What is the pathophysiology of pruritus in PV

Answer 31

consequence of mast cell activation by JAK2V617F

Question 32

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

Answer 32

True.

Question 33

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

Answer 33

True.

Question 34

most significant complication of PV

Answer 34

thrombosis

Question 35

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

Answer 35

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

Question 36

How many phlebotomy or when is it required

Answer 36

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

Question 37

serum uric acid value when allopurinol is given

Answer 37

serum uric acid of 10 mg/dL and above

Question 38

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

Answer 38

True.

Question 39

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

Answer 39

anagrelide

Question 40

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

Answer 40

False. No role

Question 41

tyrosine kinase inhibitor for PV patients unresponsive to cytotoxic agent

Answer 41

ruxolitinib

Question 42

Least common MPN

Answer 42

primary myelofibrosis

Question 43

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

Answer 43

Primary myelofibrosis