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HEM TEST 2024 > PMF facts > Flashcards

PMF facts Flashcards

1
Q

Fedratinib in 1st line PMF

A

JAKARTA
Phase III
Compared to placebo
40% SVR35 and TSS50

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2
Q

Fedratinib SVR

A

30% SVR35

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3
Q

Fedratnib SE

A

GI
Wernicke’s

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4
Q

Momelotinib SE

A

Anemia
Thrombocytopenia
Peripheral neuropathy

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5
Q

Momelotinib in 1st line PMF

A

MOMENTUM
Phase III
vs danasogood for anemia
But less for symptoms

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6
Q

Promising novel therapies in PMF

A

Pelabresib- BET inhibitor
Navitoclax
Imetelstat- telomerase inhibitor
navtemadlin- MDM2 inhibitor

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7
Q

PMF diagnosis

A

Fibrosis grade 2 accompanied by megakaryocyte atypia
Driver mutation
+ LDH/Splenomegaly/Anemia/WBC/Leukoerythroblastosis

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8
Q

Prefibrotic PMF diagnosis

A

Fibrosis G1

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9
Q

TN PMF %

A

10%

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10
Q

TN PMF prognosis

A

Poor

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11
Q

CALR1 PMF prognosis

A

Favorable

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12
Q

DIPSS in PMF

A

Age > 65
B Symp
Hb< 10
WBC> 25
Blasts>1%

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13
Q

MIPSS70 v2 in PMF

A

Not CLAR
Blasts
Anemia
Cytogenetic
Molecular

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14
Q

High molecular risk mutations in PMF

A

ASXL1, IDH1, IDH2, EZH2, SRSF2

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15
Q

Long term OS after ASCT in PMF

A

50%

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16
Q

Driver mutations % in PMF

A

JAK2 60%
CALR 20%
MPL 10%
TN 10%

17
Q

Tx of splenomegaly in low risk PMF

A

HU
PEG IFN

18
Q

Tx of anemia in PMF with Epo>500

A

Danazol
Thalidomide

19
Q

MMB minimum PLT in PMF

A

pts with PLT >25K were enrolled in the study

20
Q

Effect of ctogenetics and molecular abnormalities in post HSCT outcomes of PMF

A

Not as important
Except p53, ASXL1 and complex ctogenetics

21
Q

Effect of driver mutations in post HSCT PMF

A

MPL very favorable
>80% 5 year OS

22
Q

Effect of driver mutations in post HSCT PMF

A

MPL very favorable
>80% 5 year OS

23
Q

Splenomegaly effect on HSCT outcomes in PMF

A

Longer time to engrafment
Higher rate of fraft failure and relapse

24
Q

Use of Ruxo as maintenance after HSCT in PMF

A

Not helpful in reducing relapse
Sometimes given for post HSCT splenomegaly or GVHD

25
Q

Conditioning for PMF pts undergoing HSCT

A

RIC
With busulfan and fludarabine
MAC is not more effective

26
Q

Familial platelets disorders

A

RUNX1
ANKD26
ETV6

27
Q

Germilne disorders with predisposition to hem malignancy

A

GATA2
SAMAD9

HEM TEST 2024 (32 decks)

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