Smoldering systemic mastocytosis

Definition

A rare, slowly progressive form of systemic mastocytosis (SM) characterized by gradual accumulation of neoplastic mast cells in the visceral organs. Patients typically present with splenomegaly, hypercellular marrow and, in most cases, urticaria pigmentosa-like skin lesions.

Disease data

Classification

Disease

ORPHA code

158775

OMIM code

ICD10 code

C96.2

ICD11 code

*Soruce