SM facts Flashcards
Subtype of SM
Indolent SM
SMM
Aggressive SM
SM with associated hematologic neoplasm
Mast cell leukemia
Diseases of mast cells
Cutaneous
SM
Mast cell sarcoma
Diagnosis of SM
Mast cell Aggregates
+
KIT/
Tryptase/
>25% atypical mast cells/
CD2 or CD25 or CD30 expresion on MC
Common KIT mutation in SM
D816V
Molecular abnormalities in SM
AXL1, TET2, SRSF2, RUNX1
poor prognosis
DD of SM
Hereditary alpha tryptesemia
B findings in SM
B= “Burden”/”Bulk”
> 30% MC/Tryptase>200/KIT>10%
Organomegaly
Signs of MPN/MDS
C findings in SM
C= “Cyoteductive”/”Clinical”
Cytopenia
Liver
Spleen
GI
Bone
Blood. Heaptosplenic. GI
Clinical manifestations of SM
Anaphlaxis
Skin Rash
Bone leisons
GI
Liver
Neuropsychiatric
Cardiac- syncope
Lymphadenopathy/Splenomegaly
B symp
Cytopenia
Tx of SM
Avapritinib- PDGFRA and KIT D816V inhibitor
Midostaurin