MPNs

Question 1

4 Most common driver mutations seen in MPNs

Answer 1

JAK2 V617F
CALR
JAK2 exon 12
MPL

Question 2

WHO Diagnostic Criteria for PV

Answer 2

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

Question 3

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

Answer 3

PV

Question 4

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

Answer 4

Age >60
Thrombosis history

Question 5

How do we treat high risk PV?

Answer 5

Aspirin 81
Hydrea

Question 6

How do we treat lower risk PV?

Answer 6

low dose aspirin alone
Phlebotomy for Hct <45%

Question 6

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

Answer 6

Ruxolitinib

Question 7

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

Answer 7

Pegylated Interferon (Repeginterferon)

Question 8

Diagnostic Criteria for ET

Answer 8

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

Question 9

What are the three most common driver mutations in ET?

Answer 9

JAK2 V617F
CALR exon 9 indel
MPL exon 10 mutation

Question 10

How do we risk stratify for thrombosis in ET?

Answer 10

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

Question 11

When do we add cytoreduction for ET?

Answer 11

Thrombotic event
Age >60
Uncontrolled symptoms in low-risk

Question 12

Diagnostic criteria for primary myelofibrosis

Answer 12

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

Question 13

Treatment of asymptomatic low risk MF

Answer 13

Observation

Question 14

Treatment for low risk MF with anemia

Answer 14

ESAs

Question 15

Treatment for low risk MF with symptomatic splenomegaly

Answer 15

HU

Question 16

Treatment for int-1 risk MF with symptomatic splenomegaly

Answer 16

HU

Question 17

Treatment for asymptomatic Int-1 risk

Answer 17

Observation

Question 18

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

Answer 18

<45%

Question 19

Treatment for INt-2 MF? (2)

Answer 19

AlloSCT
Rux

Question 20

Teratment for high risk MF? (2)

Answer 20

AlloSCT
Rux

Question 21

What is the benefit of ruxolitinib in MF?

Answer 21

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

Question 22

Indication for Ruxolitinib in MF

Answer 22

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

Question 23

Indication of Fedratinib in PMF?

Answer 23

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

Question 24

Unique side effect of fedratinib

Answer 24

Wernicke's encephalopathy

Question 25

Indication for Pacritinib in MF?

Answer 25

Platelets <50k

Question 26

What is an unfavorable karyotype in PMF?

Answer 26

Any karyotype that is not normal

Question 27

Mutation associated with systemic mastocytosis

Answer 27

KIT D816V

Question 28

Treatment for KIT D618V mutation systemic mastocytosis (2)

Answer 28

Midostaurin Avapritinib

Question 29

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

Answer 29

Imatinib

Question 30

Diagnostic criteria for Chronic Neutrophilic Leukemia (4)

Answer 30

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

Question 31

Treatment for CNL?

Answer 31

Ruxolitinib

Question 32

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

Answer 32

CALR

Question 33

Treatment for Hypereosinophilic syndrome with PDGFRA/B rearrangement?

Answer 33

Imatinib

Question 34

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

Answer 34

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

Question 35

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

Answer 35

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

Question 36

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

Answer 36

Vemurafenib

Question 37

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

Answer 37

Cobimetinib

Question 38

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

Answer 38

Cytarabine or cladribine

Question 39

Preferred treatment for Langerhans cell histiocytosis with bone only disease

Answer 39

Bisphosphonete

Question 40

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

Answer 40

MTX Hydrea

Question 41

Treatment for high risk ET pregnant patient?

Answer 41

ASA Peginterferon alfa 2a

Question 42

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

Answer 42

Peginterferon Anagrelide

Question 43

1L treatment for indolent systemic mastocytosis

Answer 43

Avapritinib

Question 44

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

Answer 44

Momelotinib