JMML Young Ewog

Which of the following signs is RARELY observed in JMML

• splenomegaly

• cough

• CNS infiltration

• rash

• fever

Which of the following PB/BM smear pairs is more likely to correspond to the JMML patient?

• A

• B

• C

• D

• E

Which of these features are considered high risk?

• WBC 35x10e9

• Plt 53x10e9

• HbF 23%

• 4% of monoblasts

What single test will be more helpful at this stage?

• Karyotyping 

• GM-CSF cell culture

• Molecular diagnosis of Ras pathway mutations and BCR/ABL exclusion

• Methylation profiling

Which of these statments about somatic PTPN11 is FALSE

• it is the most common mutation in JMML

• usually has lower age of presentation

• is often self-limiting, "watch and wait“ strategy is possible

• usually found in high methylation group

What would be your first therapeutic intervention in this case?

• Upfront HSCT without prior therapy

• AML-like induction therapy 

• Fludarabine and cytarabine

• 6 Mercaptopurine

•  Azacitidine

How would you condition this patient if a MUD 10/10 is available?

• Treosulfan, Cyclophosphamide and Melphalan

• Busulfan, Cyclophosphamide and Melphalan

• Busulfan and Fludarabine

• Busulfan, Fludarabine and Melphalan

• Treosulfan, Fludarabine and Melphalan