Quiz: What Is the Only Curative Treatment for Myelofibrosis?

Which genetic mutation confers shorter progression-free survival in myelofibrosis patients undergoing allogeneic stem cell transplantation? And what is the only potentially curative treatment for primary myelofibrosis? Test your knowledge in our latest quiz.

Question 1

Answer

B.CALR

According to NCCN guidelines, the 2016 WHO diagnostic criteria includes molecular testing for CALR, JAK2, and MPL mutations for essential thrombocytosis and primary myelofibrosis, and JAK2 V617F or JAK2 exon 12 mutations for patients with polycythemia vera.

Question 2

Answer

A. Essential thrombocythemia

The main diagnostic criteria for essential thrombocythemia are the proliferation of bone marrow megakaryocytic, thrombocytosis, and the presence of either a CALR, JAK2,or MPL mutation.

Question 3

Answer

D.Ruxolitinib

The oral drug ruxolitinib, a JAK1/2 inhibitor, is currently the only agent approved for the treatment of myelofibrosis.

Question 4

Answer

C.Allogeneic stem cell transplantation

Despite the approval of ruxolitinib, allogeneic stem cell transplantation remains the only curative therapy for myelofibrosis.

Question 5

Answer

C. IDH2

According to a study published in Biology of Blood and Marrow Transplantation, both IDH2 and ASXL1 mutations were independent risk factors for shorter progression-free survival among patients undergoing allogeneic stem cell transplantation for myelofibrosis, with hazard ratios of 5.451 for IDH2 (P = .002) and 1.53 for ASXL1 (P = .008).