How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

Abstract

Indolent systemic mastocytosis (SM) flow-cytometric diagnostic methods are patients have a varied clinical presenta-very sensitive and can detect minimal intion, ranging from predominantly cutane-volvement of bone marrow with atypical/ ous symptoms to recurrent systemic clonal mast cells; in some cases, full symptoms (eg, flushing, palpitations, diagnostic criteria for SM are not fulfilled. dyspepsia, diarrhea, bone pain) that can An important aspect of treatment is be severe and potentially life threatening avoidance of known symptom triggers; (anaphylaxis). Mastocytosis patients other treatment principles include a step-without skin involvement pose a diagnos-wise escalation of antimediator theratic challenge; a high index of suspicion pies and consideration of cytoreductive is needed in those with mast cell–therapies for those with treatment-degranulation symptoms, including ana-refractory symptoms. The perioperative phylaxis following Hymenoptera stings or management of mastocytosis patients is other triggers. Modern-era molecular and nontrivial; a multidisciplinary preoperative assessment, adequate premedications, and close intra- and postoperative monitoring are critical. Smoldering mastocytosis is a variant with high systemic mast cell burden. While its clinical course can be variable, there is greater potential need for cytoreductive therapies (eg, interferon-alpha, cladribine) in this setting. A systematic approach to the diagnosis and treatment of indolent SM using a case-based approach of representative clinical scenarios is presented here.