Polycythemia vera Flashcards
Driver mutation in p vera
JAK2 V617 (96%)
Second most common mutation in p vera
JAK2 EXON 12
clinical features of p vera
- low serum EPO (typically) + splenomegaly + elevated leukocyte and platelet count + erythromelalgia.
Management of polycythemia in a post renal transplant patient
ACE inhibitors
Tempi syndrome clinical features
high EPO + monoclonal gammopathy
What is considered high risk PV?
> 60 yo OR VTE history
low risk PV management
Phlebotomy alone (Preferred – Phlebotomy safer than hydrea. Better to avoid SE’s of hydrea though well tolerated, and to prevent other cytopenias)
IF uncontrolled PV-associated symptoms OR progressive increase of leukocyte and/or platelet counts OR symptomatic or progressive splenomegaly OR Poor tolerance of phlebotomy, hydroxyurea
high risk PV management
- anticoagulation if VTE history
- hydrea
- ASA 81 mg
Indications for hydrea in low risk PV
- uncontrolled PV-associated symptoms with phlebotomy
- progressive increase of leukocyte and/or platelet counts
- symptomatic or progressive splenomegaly
- Poor tolerance of phlebotomy
Management of pregnant PV patient
Ropeginterferon (pegylated INF-alpha)
1) Second line options for PV 2) Preferred second line option
Jakafi (Preferred)
*Busulfan
Anagrelide
*Ropeginterferon also an option second line?
Management of post PV AML
Allo if transplant eligible
