MPN

Question 1

What are examples of clonal driver mutations seen in CHIP?

Answer 1

• TET2
• DNMT3A

Question 2

Low risk vs. high risk PV

Answer 2

• in low risk, just ASA + phlebotomy
• high risk patients need cytoreduction

Question 3

Preferred cytoreduction for age less than 40

Answer 3

interferon vs. ropeginterferon

Question 4

preferred cytoreductive drug for PV patients intolerant

Answer 4

jakafi

Question 5

Contraindications to interferon

Answer 5

depression or autoimmune disease

Question 6

What does risk stratification tool for thrombosis in ET include?

Answer 6

• age over 60
• CV risk factors
• previous VTE
• JAK2V617 (more thrombogenic)

Question 7

When is cytoreduction indicated in ET?

Answer 7

• only in high risk (like in PV)

Question 8

Low risk MF management

Answer 8

• observation if asymptomatic
• ESA if anemic
• lenalidomide if del(5q)
• hydrea if symptomatic splenomegaly

Question 9

Jakafi mechanism

Answer 9

JAK1 + JAK2 inhibitor

Question 10

mutation to know for systemic mastocytosis

Answer 10

KIT D816V

Question 11

mastocytosis clinical features

Answer 11

• flushing, pruritus, urticaria
• GI tract involvement
• lymphadenopathy + splenomegaly

Question 12

drugs approved for systemic mastocytosis

Answer 12

• midostaurin
• avapritinib

Question 13

CNL treatment

Answer 13

jakafi