Systemic Mastocytosis

Madhuri Yalamanchili, Anamika Patel, P. Bergevin
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Abstract

Systemic Mastocytosis is an abnormal proliferation of mast cells in the skin and multiple other organs. Its prognosis is variable, from indolent to very aggressive. The systemic signs and symptoms of mastocytosis are diverse and related to pharmacologic manifestations of mast cell mediators. They may mimic various conditions including chronic granulomatous disorders such as tuberculosis and sarcoid, connective tissue disorders, occult visceral malignancies, and even lymphoma. We report a patient with systemic mastocytosis and review the diagnosis and management of the disorder.
系统性肥大细胞增多症是肥大细胞在皮肤和其他多个器官中的异常增生。它的预后是可变的,从无痛到非常严重。肥大细胞增多症的全身体征和症状多种多样,并与肥大细胞介质的药理学表现有关。它们可以模拟各种情况,包括慢性肉芽肿疾病,如结核和肉瘤,结缔组织疾病,隐性内脏恶性肿瘤,甚至淋巴瘤。我们报告一个系统性肥大细胞增多症的病人,并回顾诊断和管理的障碍。
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