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Malignant Hematology > MPNs > Flashcards

MPNs Flashcards

1
Q

4 Most common driver mutations seen in MPNs

A

JAK2 V617F
CALR
JAK2 exon 12
MPL

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2
Q

WHO Diagnostic Criteria for PV

A

Hb >16.5 for men or >16 in women OR
Hct >49% or 48%
Presence of JAK2 V617F mutation (or other similar mut)
Hypercellular BM with panmyelosis

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3
Q

Hypercellular Bone marrow with panmyelosis and a JAK2 V617F mutation is suggestive of which MPN?

A

PV

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4
Q

What makes a patient with PV high risk? (2)

A

Age >60
Thrombosis history

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5
Q

How do we treat high risk PV?

A

Aspirin 81
Hydrea

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6
Q

How do we treat lower risk PV?

A

low dose aspirin alone
Phlebotomy for Hct <45%

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6
Q

How do we treat high risk PV that is intolerant or resistant to hydrea?

A

Ruxolitinib

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7
Q

What is another cytoreductive treatment option for high risk PV aside from hydrea?

A

Pegylated Interferon (Repeginterferon)

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8
Q

Diagnostic Criteria for ET

A

Sustained platelet >450
Megakaryocytic hyperplasia on BM
Presence of JAK2, CALR, or MPL mut
Not meeting criteria for other MPN

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9
Q

What are the three most common driver mutations in ET?

A

JAK2 V617F
CALR exon 9 indel
MPL exon 10 mutation

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10
Q

How do we risk stratify for thrombosis in ET?

A

Age >60 (1)
CV RFs (1)
Previous thrombosis (2)
JAK2 V617F (2)
Low risk = 0-1
Intermediate = 2
High risk = 3+

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11
Q

When do we add cytoreduction for ET?

A

Thrombotic event
Age >60
Uncontrolled symptoms in low-risk

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12
Q

Diagnostic criteria for primary myelofibrosis

A

Major: (need all)
Megakaryocytic proliferation with reticulin/collagen fibrosis
Presence of JAK2, CALR, or MPL mutation or another clonal marker
Not meeting WHO criteria for other MPN
Minor: (need 1)
Anemia
Leukocytosis
Palpable splenomegaly
LDH > ULN
Leukoerythroblastosis

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13
Q

Treatment of asymptomatic low risk MF

A

Observation

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14
Q

Treatment for low risk MF with anemia

A

ESAs

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15
Q

Treatment for low risk MF with symptomatic splenomegaly

A

HU

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16
Q

Treatment for int-1 risk MF with symptomatic splenomegaly

A

HU

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17
Q

Treatment for asymptomatic Int-1 risk

A

Observation

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18
Q

What is the goal Hct for patients with PV who are undergoing phlebotomy?

19
Q

Treatment for INt-2 MF? (2)

A

AlloSCT
Rux

20
Q

Teratment for high risk MF? (2)

A

AlloSCT
Rux

21
Q

What is the benefit of ruxolitinib in MF?

A

Improves splenomegaly and constitutional symptoms, improves OS. in intermediate/high risk MF

22
Q

Indication for Ruxolitinib in MF

A

Int/high risk MF regardless of JAK2 mutation status
Plt >50k
Not transplant candidate

23
Q

Indication of Fedratinib in PMF?

A

1L or Rux refractory, Plt >50k
Not transplant candidate

24
Q

Unique side effect of fedratinib

A

Wernicke’s encephalopathy

25
Q

Indication for Pacritinib in MF?

A

Platelets <50k

26
Q

What is an unfavorable karyotype in PMF?

A

Any karyotype that is not normal

27
Q

Mutation associated with systemic mastocytosis

28
Q

Treatment for KIT D618V mutation systemic mastocytosis (2)

A

Midostaurin
Avapritinib

29
Q

KIT 816V mutation in systemic mastocytosis renders resistance to what treatment?

30
Q

Diagnostic criteria for Chronic Neutrophilic Leukemia (4)

A

WBC 25+
Hypercellular BM
Presence of CSF3R mutation
No rearrangement in PDGFRA, B, FGFR1, or PCM1-JAK2

31
Q

Treatment for CNL?

A

Ruxolitinib

32
Q

Which driver mutation of MPNs confers a better prognosis compared to JAK2 or triple negative?

33
Q

Treatment for Hypereosinophilic syndrome with PDGFRA/B rearrangement?

34
Q

What makes up the Dynamic IPI for MF? (5)

A

Age >65 (1)
Constitutional sx (1)
Hb <10 (2)
WBC >25 (1)
Blasts 1%+ (1)

35
Q

What are cutoffs for low, Int-1, int-2, and high risk in the dynamic IPI for MF?

A

Low = 0
Int-1 = 1-2
Int-2 = 3-4
High = 5-6

36
Q

Treatment for multisystem or single system lung-Langerhans cell Histiocytosis with BRAF V600E mutation

A

Vemurafenib

37
Q

Treatment for multisystem or single system lung-Langerhans cell Histiocytosis with MAPK pathway mutation

A

Cobimetinib

38
Q

Treatment for multisystem or single system lung-Langerhans cell Histiocytosis without BRAF or MAPK pathway mutation (2)

A

Cytarabine or cladribine

39
Q

Preferred treatment for Langerhans cell histiocytosis with bone only disease

A

Bisphosphonete

40
Q

Preferred treatment for Langerhans cell histiocytosis with multifocal skin disease (2)

41
Q

Treatment for high risk ET pregnant patient?

A

ASA
Peginterferon alfa 2a

42
Q

Treatment for those with high risk ET and inadequate response to hydrea? (2)

A

Peginterferon
Anagrelide

43
Q

1L treatment for indolent systemic mastocytosis

A

Avapritinib

44
Q

Teratment for intermediate/high risk MF who are not transplant candidates and have primarily anemia problems

A

Momelotinib

Malignant Hematology (13 decks)

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