MPNs and MDS

Question 1

Philadelphia chromosome

Answer 1

t(9;22)

Question 2

bcr-abl in ALL->

bcr-abl in CML->

Answer 2

ALL-p190

CML-p210

Question 3

Definition of complete hematologic response->
Definition of complete cytogenetic response->
Definition of major molecular response->

Answer 3

CHR-> 90% reduction at 3 months
CCyR-> 2 log reduction in transcript at 12 months
MMR-> 3 log reduction by 18 mos

Question 4

TKI which can cause pancreatitis and arterial thrombosis

Answer 4

Ponatinib

Question 5

TKI associated with pleural effusions and pulmonary hypertension

Answer 5

Dasatinib

Question 6

TKI associated with QT prolongation, prurirtis and skin rash

Answer 6

Nilotinib

Question 7

TKI associated with diarrhea, rash, and elevated LFTs

Answer 7

Bosutinib

Question 8

TKI needed for F317L mutation in CML (3 options)

Answer 8

Nilotinib, Bosutinib, Omacetaxine

Question 9

c-kit mutation to know in Systemic mastocytosis

Answer 9

Asp816Val. This will not respond to imatinib and needs to be treated with midostaurin

Question 10

Mutation which suggests response to imatinib in CMML

Answer 10

t (5;12). fusion between TEL gene and PDGFR

Question 11

What cardiac effect can be seen in patients with chronic eosinophilic leukemia

Answer 11

Endomyocardial fibrosis resulting in a restrictive cardiomyopathy (constrictive pericarditis)

Question 12

Highest risk ET

Answer 12

Age >60
JAK2 +
Hx of VTE

They need cytoreduction and ASA

Question 13

What gene mutation is present in Chuvash Polycythemia?

Answer 13

VHL mutation. Leads to defecetive oxygen sensing and secondary EPO overproduction

Question 14

5 risk factors in PMF

Answer 14

Age > 65
Constitutional Symptoms
Blood blasts >1%
WBC > 25k
Hgb <10

> 3 is highest risk with median survival 30 months

Question 15

syndrome associated with fedratinib

Answer 15

Wenicke’s. Give thiamine ahead of time.

Question 16

Very Good (2) and Good (4) Risk MDS cytogenetics

Answer 16

Very Good-> -Y, Del 11q

Good-> Normal, -5q, -20q, -12p

Question 17

Poor (2) and Very Poor (3) Risk MDS Cytogenetics

Answer 17

Poor-> -7, i3/-3q

Very Poor-> Complex karyotype, TERT/p53 mutations

Question 18

Mutation in congenital sideroblastic anemia

Answer 18

ALAS2. Does not progress to AML

Question 19

Mutation which can suggest higher resposne rate to hypomethylating agents in MDS

Answer 19

TET2 mutation

Question 20

How do you differentiated hypocellular MDS from aplastic anemia

Answer 20

HLA-DR15 + is seen in aplastic anemia. Responds to IS