Myelofibrosis

Question 1

Other sites of extramedullary hematopoiesis

Answer 1

lungs (can lead to pleural effusion)
paraspinal (can put pts at risk for cord compression)

Question 2

WHO major diagnostic criteria for PMF

Answer 2

1) megakaryocyte proliferation and atypia accompanied by reticulin and collagen fibrosis
2) Not meeting diagnostic criteria for other disorders
3) JAK2, CALR, or MPL mutant

Question 3

Secondary causes of myelofibrosis

Answer 3

AML →
Late stage PV/ET/CML →
Infection →
HCL →
Autoimmune myelofibrosis (AIMF) vs. PMF →

Question 4

Mutation with better prognosis in PMF

Answer 4

CALR

Question 5

Mutational status with worst prognosis in PMF

Answer 5

triple negative

Question 6

High molecular risk mutations in PMF

Answer 6

• ASXL1
• IDH1/2
• EZH2
• SRSF2

Question 7

Adverse prognostic features in PMF incorporated into DIPSS

Answer 7

• age over 65
• symptoms
• high white count (greater than 25) and blast count
• anemia, thrombocytopenia

Question 8

Chromosomal abnormalities associated with poor prognosis

Answer 8

1) +8
2) -7/7q-
3) i(17q)
4) -5/5q-
5) 12p-
6) inv(3)
7) 11q23

Question 9

Low risk PMF management

Answer 9

Given asymptomatic, plan for active surveillance

Question 10

JAK inhibitor with less effect on platelet count

Answer 10

pacritinib

Question 11

Continuation of JAK inhibitor

Answer 11

if no improvement in spleen size or symptoms after 6 months supposed to discontinue (taper)

Question 12

Management of encephalopathy from fedratinib

Answer 12

• thiamine (it’s wernicke’s)

Question 13

Platelet count preclude ruxolitinib and fedratinib

Answer 13

50k

Question 14

Management of accelerated/higher risk PMF

Answer 14

• Transplant if candidate
• HMA
  +/- jaki

Question 15

Factors included in DIPSS

Answer 15

• Age >65 years: 1 point
• Leukocyte count >25,000/microL (>25 x 109/L): 1 point
• Hemoglobin <10 g/dL (<100 g/L): 2 points
• Circulating blast cells ≥1%: 1 point
• Constitutional symptoms*: 1 point