SM facts Flashcards

1
Q

Subtype of SM

A

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

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2
Q

Diseases of mast cells

A

Cutaneous
SM
Mast cell sarcoma

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3
Q

Diagnosis of SM

A

Mast cell Aggregates
+
KIT/
Tryptase/
>25% atypical mast cells/
CD2 or CD25 or CD30 expresion on MC

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4
Q

Common KIT mutation in SM

A

D816V

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5
Q

Molecular abnormalities in SM

A

AXL1, TET2, SRSF2, RUNX1
poor prognosis

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6
Q

DD of SM

A

Hereditary alpha tryptesemia

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7
Q

B findings in SM

A

B= “Burden”/”Bulk”

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

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8
Q

C findings in SM

A

C= “Cyoteductive”/”Clinical”

Cytopenia
Liver
Spleen
GI
Bone

Blood. Heaptosplenic. GI

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9
Q

Clinical manifestations of SM

A

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

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10
Q

Tx of SM

A

Avapritinib- PDGFRA and KIT D816V inhibitor
Midostaurin

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