SM facts

Question 1

Subtypes of SM

Answer 1

Indolent SM
SMM
Aggressive SM
SM with associated hematologic neoplasm
Mast cell leukemia

Question 2

Diseases of mast cells

Answer 2

Cutaneous
SM
Mast cell sarcoma

Question 3

Diagnosis of SM

Answer 3

Mast cell Aggregates
+
KIT/
Tryptase/
>25% atypical/spindel shaped mast cells/
CD2 or CD25 expresion on MC

Question 4

Common KIT mutation in SM

Answer 4

D816V

Question 5

Molecular abnormalities in SM

Answer 5

AXL1, TET2, SRSF2, RUNX1
poor prognosis

Question 6

DD of SM

Answer 6

Hereditary alpha tryptasemia

Question 7

B findings in SM

Answer 7

B= “Burden”/”Bulk”

> 30% MC/Tryptase>200/KIT>10%
Organomegaly
Signs of MPN/MDS

Question 8

C findings in SM

Answer 8

C= “Cyoteductive”/”Clinical”

Cytopenia
Liver
Spleen
GI
Bone

Blood. Heaptosplenic. GI

Question 9

Clinical manifestations of SM

Answer 9

Anaphlaxis
Skin Rash
Bone leisons
GI
Liver
Neuropsychiatric
Cardiac- syncope
Lymphadenopathy/Splenomegaly
B symp
Cytopenia

Question 10

Tx of SM

Answer 10

Avapritinib- PDGFRA and KIT D816V inhibitor
Midostaurin