PMF facts Flashcards
Fedratinib in 1st line PMF
JAKARTA
Phase III
Compared to placebo
40% SVR35 and TSS50
Fedratnib SE
GI
Wernicke’s
Momelotinib SE
Anemia
Thrombocytopenia
Peripheral neuropathy
Momelotinib in 1st line PMF
MOMENTUM
Phase III
vs danasol
Good for anemia
But less for symptoms
PLT > 25K were enrolled
Promising novel therapies in PMF
Pelabresib- BET inhibitor
Navitoclax
Imetelstat- telomerase inhibitor
PMF diagnosis
Fibrosis grade 2 accompanied by megakaryocyte atypia
Driver mutation
+ LDH/Splenomegaly/Anemia/WBC/Leukoerythroblastosis
Prefibrotic PMF diagnosis
Fibrosis G1
TN PMF %
10%
TN PMF prognosis
Poor
CALR1 PMF prognosis
Favorable
DIPSS in PMF
Age > 65
Const symptoms
Hb< 10
WBC> 25
Blasts>1%
MIPSS70 v2 in PMF
Not CLAR
Blasts
Anemia
Cytogenetic (poor- -7, i17, -3, -11, -12. Favorable- 9, 13q, 20, Y)
Molecular (ASX1, U2AF1, SRSF2, IDH1, IDH2, EZH2)
“AUSIIE”
High molecular risk mutations in PMF
ASXL1, IDH1, IDH2, EZH2, SRSF2, U2AF1
Long term OS after ASCT in PMF
50%
Driver mutations % in PMF
JAK2 60%
CALR 20%
MPL 10%
TN 10%
Tx of splenomegaly in low risk PMF
JAK2
HU
PEG IFN
Tx of anemia in PMF with Epo>500
Danazol
Thalidomide+prednisone
MMB minimum PLT in PMF
pts with PLT >25K were enrolled in the study
Effect of ctogenetics and molecular abnormalities in post HSCT outcomes of PMF
Not as important
Except p53, ASXL1 and complex ctogenetics
Effect of driver mutations in post HSCT PMF
MPL very favorable
>80% 5 year OS
Splenomegaly effect on HSCT outcomes in PMF
Longer time to engrafment
Higher rate of graft failure and relapse
Use of Ruxo as maintenance after HSCT in PMF
Not helpful in reducing relapse
Sometimes given for post HSCT splenomegaly or GVHD
Conditioning for PMF pts undergoing HSCT
RIC
With busulfan and fludarabine + thiotepa
MAC is not more effective
DIPSS vs DIPSS+ in PMF
+
Cytogenetics
PLT<100K
Transfusion dependance
