Aggressive systemic mastocytosis: a case report and brief review of the literature.

Abstract

Aggressive Systemic Mastocytosis (ASM), a rare subtype of Systemic Mastocytosis (SM), results from clonal proliferation and invasion of multiple organs by neoplastic mast cells. The clinical presentation varies, dependent on which organ systems are involved, and may take an indolent or rapidly fatal course. Several treatment strategies have been proposed. The most effective treatment, though not curative, is a combination of glucocorticoids and alpha-interferon 2b (IFN-alpha). We present a case report of ASM to demonstrate the clinical presentation, laboratory findings, and therapeutic response to treatment.