Question 1

What is the primary mutation associated with Primary Myelofibrosis (PMF)?

Accepted Answer

JAK2

Answer

BCR-ABL

Answer

NPM1

Answer

FLT3

Answer

KRAS

Question 2

Which cytokine contributes to fibroblast formation and collagen deposition in PMF?

Accepted Answer

TGF-β

Answer

IFN-γ

Answer

VEGF

Answer

TNF-α

Answer

IL-2

Question 3

What percentage of PMF cases are associated with JAK2 mutations?

Accepted Answer

50-60%

Answer

40-50%

Answer

70-80%

Answer

20-30%

Answer

90-100%

Question 4

What is a key diagnostic feature of overtly fibrotic PMF?

Accepted Answer

≥ Grade 2 reticulin/collagen fibrosis

Answer

Hypocellular bone marrow

Answer

Lack of anemia

Answer

Grade 1 reticulin fibrosis

Answer

Normal spleen size

Question 5

What is the most frequent cytogenetic abnormality seen in PMF?

Accepted Answer

Del(20q)

Answer

Duplication of 1q

Answer

FLT3 mutation

Answer

T(9;22) translocation

Answer

Trisomy 21

Question 6

What does the GIPSS prognostic model for PMF incorporate?

Accepted Answer

Cytogenetics and mutations

Answer

Red cell transfusion requirement

Answer

Presence of anemia

Answer

Platelet count

Answer

Age at diagnosis

Question 7

Which karyotype is considered favorable in PMF?

Accepted Answer

Isolated del(20q)

Answer

Complex karyotype

Answer

Monosomy 7

Answer

Trisomy 8

Answer

T(9;22) translocation

Question 8

What is the key difference between prefibrotic PMF and essential thrombocythemia (ET)?

Accepted Answer

Morphology of megakaryocytes

Answer

Mutations in FLT3

Answer

Platelet count

Answer

Levels of hemoglobin

Answer

Presence of leukopenia

Question 9

What is the typical bone marrow finding in the overt stage of PMF?

Accepted Answer

Alternating cellular and hypocellular areas

Answer

Hypocellular marrow

Answer

Eosinophilic infiltrates

Answer

Hypercellular bone marrow

Answer

Diffuse marrow replacement by fibrosis

Question 10

Which stain is commonly used to detect reticulin fibers in PMF?

Accepted Answer

Masson’s trichrome

Answer

Prussian blue

Answer

Periodic acid-Schiff (PAS)

Answer

Giemsa

Answer

Hematoxylin and eosin (H&E)

Question 11

Which mutation is commonly associated with worse prognosis in PMF?

Accepted Answer

ASXL1 mutation

Answer

TP53 mutation

Answer

NRAS mutation

Answer

CALR mutation

Answer

MPL mutation

Question 12

Which feature distinguishes PMF from other myeloproliferative neoplasms (MPNs) like essential thrombocythemia (ET)?

Accepted Answer

Tight clustering of atypical megakaryocytes with hyperchromatic nuclei

Answer

Elevated lactate dehydrogenase (LDH) levels

Answer

Hemoglobin levels below 10 g/dL

Answer

Occurrence of JAK2 mutations in >95% of cases

Answer

Platelet count >1 million/µL

Question 13

What is the molecular role of CALR mutations in PMF pathogenesis?

Accepted Answer

Constitutive activation of the thrombopoietin receptor (MPL)

Answer

Inhibition of erythropoiesis

Answer

Activation of neutrophil elastase

Answer

Dysregulation of TP53

Answer

Decreased IL-1 production

Question 14

Which prognostic factor is unique to the MIPSS70+ version 2.0 (MIPSSv2) model for PMF?

Accepted Answer

Incorporation of high-risk mutations such as ASXL1, SRSF2, and U2AF1

Answer

Platelet count <100 × 10⁹/L

Answer

Presence of constitutional symptoms

Answer

Age above 65 years

Answer

Blast percentage in the bone marrow

Question 15

What is the most frequent cause of death in patients with PMF?

Accepted Answer

Transformation to acute myeloid leukemia (AML)

Answer

Pulmonary hypertension

Answer

Heart failure

Answer

Thromboembolic events

Answer

Solid organ rupture (spleen or liver)

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