Isolated bone marrow mastocytosis

Definition

A rare subtype of indolent systemic mastocytosis characterized by isolated bone marrow involvement without skin lesions, low burden of neoplastic mast cells, and often normal or near normal serum tryptase levels. The <i>KIT</i> D816V mutation is present in the majority of cases.

Disease data

Classification

Disease

ORPHA code

158778

OMIM code

ICD10 code

D47.0

ICD11 code

2A21.0Y

*Soruce