Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Poland.
Arch Med Sci. 2012 Jul 4;8(3):533-41. doi: 10.5114/aoms.2012.29409.
Mastocytosis is a clonal disease of the hematopoietic stem cell. The condition consists of a heterogeneous group of disorders characterized by a pathological accumulation of mast cells in tissues including the skin, bone marrow, liver, spleen and the lymph nodes. Mastocytosis is a rare disease which occurs both in children and adults. Childhood onset mastocytosis is usually cutaneous and transient while in adults the condition commonly progresses to a systemic form. The heterogeneity of clinical presentation of mastocytosis is typically related to the tissue mast cell burden, symptoms due to the release of mast cell mediators, the type of skin lesions, the patient's age at the onset and associated haematological disorders. Therefore, a multidisciplinary approach is recommended. The present article provides an overview of clinical symptoms, diagnostic criteria and treatment of mastocytosis to facilitate the diagnosis and management of mastocytosis patients in clinical practice.
肥大细胞增多症是造血干细胞的克隆性疾病。其特征为组织中肥大细胞病理性堆积，包括皮肤、骨髓、肝、脾、淋巴结等，由一组异质性疾病组成。肥大细胞增多症为罕见疾病，可发生于儿童及成人。儿童发病的肥大细胞增多症通常为皮肤型且为一过性，而在成人，该疾病通常进展为系统性疾病。肥大细胞增多症的临床表现异质性通常与组织中肥大细胞负荷、肥大细胞介质释放引起的症状、皮肤病变类型、发病时患者年龄以及相关血液学疾病相关。因此，推荐采用多学科方法。本文概述了肥大细胞增多症的临床表现、诊断标准和治疗方法，旨在促进肥大细胞增多症患者的临床诊断和管理。
