MDS/MPN facts

Question 1

CNL Tx

Answer 1

Hydrea/IFN
Ruxo
HMA

Question 2

Gene rearrangements in M/L neoplasms with eosinophilia

Answer 2

PDGFRA, PDGFRB, FGFR1, JAK2, FLT3, ETV6::ABL1

Question 3

Clonal disorder in eosinophilia workup %

Answer 3

10%

Question 4

MLN- TK with PDGFRA/PDGFRB rearrangement Tx

Answer 4

Imatinib
A 100 mg
B 400 mg

Question 5

MLN- TK with FGFR1 rearrangement Tx

Answer 5

Pemigatinib
FGFR1, 2 and 3 inhibitor

Question 6

MLN- TK with ETV6::ABL1 rearrangement Tx

Answer 6

Dasatinib
Nilotinib
Imatinib

Question 7

Diagnosis of MDS/MPN-U

Answer 7

not meeting criteria for MDS, MPN
clinical features of MDS with WBC> 13 and PLT>45O

Question 8

Blastic plasmacytoid dendritic cell neoplasm

Answer 8

Agressive neoplasm with skin lesions
May develop to AML/CMML
Tagraxofusp- anti IL3 (conjugated)

Question 9

Tagraxofusp

Answer 9

anti IL3 (conjugated)
Tx of BPDCN

Question 10

aCML BMB findings

Answer 10

Hypercellularity
Fibrosis (more than CML)
Dysgranulopoiesis
No eosinophilia/basophilia

Question 11

aCML diagnosis

Answer 11

WBC> 13K
>10% circulating immature cells
balsts< 20%
Prominent dysgranulopoiesis
monocytes, eosinophiles< 10%
BCR:ABL1, and other driving mutations negative
ASXL1 and SETBP1 positive

Question 12

Most common cytogenetic abnormalities in aCML

Answer 12

+8
-7

Question 13

Most common mutations in aCML

Answer 13

ASXL1 (60–90%)
SETBP1
ETKN1
TET2
SRSF2

Question 14

Prognostic risk factors for aCML

Answer 14

age> 67
Hb< 10
TET2 mutation

Question 15

Median OS for aCML

Answer 15

10-30 months

Question 16

Incidence of aCML

Answer 16

1-2% of CML

Question 17

MDS/MPN-NOS

Answer 17

diagnosis not meeting any MPN
mutation profile can devide to:
CMML like
aCML like
MDS/MPN-ring sideroblasts-thrombocytosis like
p53 +
and other
prognosis and Tx is based on genetic profiling

Question 18

Langerhans cell histiocytosis catagorization

Answer 18

Single system
Multisystem

Question 19

Single system Langerhans cell histiocytosis

Answer 19

Bone- most common
Skin
Lymph nodes
Lung

Question 20

Tx of bone involvement of LCH

Answer 20

Resection/radiation/steroid injection

Question 21

Tx of skin involvement of LCH

Answer 21

Topical steroids
Nitrogen mustard cream
Phototherapy

Question 22

Tx of lung involvement of LCH

Answer 22

Smoking cessation
Prednisone, vinblastine, MTX
Immunosuppressive agents.

Question 23

Erdheim-Chester disease clinical manifestations

Answer 23

Osteosclerosis of
the legs
Fibrosis of skeleton, retroperitoneum,
skin, CNS, heart, lungs, and less commonly, lymph nodes,
liver, and spleen

Question 24

Tx of Erdheim-Chester disease

Answer 24

Interferon-a

Question 25

Rosai-Dorfman disease

Answer 25

Sinus histiocytosis with massive lymphadenopathy

Question 26

HLH criteria

Answer 26

Fever
Splenomegaly
Cytopenias
Hemophagocytosis on BM
High IL2
Ferritin
Triglycerides
Low NK

Question 27

MDS-MPN NOS criteria

Answer 27

MDS Features
with WBC>13/ PLT>450

Question 28

Tx of multisystem langerhans cell histiocytosis

Answer 28

Vinblastine + prednisone

Question 29

Hypereosinophilia definition

Answer 29

> 1500 (at least 1 month)
And 10%

Question 30

Hypereosinophilic syndrome definition

Answer 30

Eosinophilia
With clinical manifestations

Question 31

Severe eosinophilia

Answer 31

> 5K

Question 32

Tx of hyperosinophilc syndrome (HES)

Answer 32

Steroids
Anti-IL5 (mepolizumab)