Niloofar Ghodrati

Mastocytosis comprises a heterogeneous group of disorders characterized by expansion and accumulation of neoplastic mast cells(MCs)in1 or more organ systems. In patients with systemic mastocytosis(SM), neoplastic MCs form focal and/ordiffuse infiltrates in various internal organs, including the bone marrow (BM), spleen, liver, and gastrointestinal tract. Regardless of the type of SM, the BM is involved in virtually all patients.Skin involvement is usually found inpatients with indolent SM(ISM),is less frequently detected in aggressive SM(ASM),and is rarely seen in MC leukemia (MCL). Based on previous classification proposals and the classification of the World Health Organization (WHO), mastocytosis can be divided into subvariants of cutaneous mastocytosis (CM), in which no systemic involvement is found, systemic variants (SM), and localized MC tumors.Systemic mastocytosis is defined as a clonal disorder of mast cells and associated with significant increase in mast cell number in skin and internal organs.its clinical course has a wide spectrum, ranging from indolent disease with normal life expectancy, to highly aggressive disease,associated with multisystemic involvement and poor prognosis. The aim of this study to report of a case of systemic mastocytosis.Case report: A 34 years old woman with LUQ pain and weakness from one yerar ago and a history of thrombocytopenia came to our clinic .she had episods of headache and flushing , thachicardia that became better after vomiting. Sometimes these symptoms was associated with using of IBUPROFEN. also she had easy brusing and epistaxy and a history of skin rash from seven year ago . In physical examination she had huge splenomegaly and hyperpigmented macul in body and trunks.she underwent bone marrow aspiration and biopsy and after second opinion and immunohistochemical staining reveald the diagnosis of systemic mastocytosis