Schmidt M, Dercken C, Loke O, Reimann S, Diederich S, Blasius S, Heidenreich S
Dept of Medicine, University of Münster, Germany.
Eur Respir J. 2000 Mar;15(3):623-5. doi: 10.1034/j.1399-3003.2000.15.32.x.
Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon alpha-2a over 6 months, the patient's condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.
系统性肥大细胞疾病是一种病因不明的罕见疾病。肥大细胞在皮肤、骨髓、胃肠道和淋巴结的系统性浸润和增殖是其主要病理特征。本研究报告了一名患有皮肤肥大细胞增多症（色素性荨麻疹）10年的患者。该患者因呼吸困难入院。胸部X线片显示双肺中度网状浸润，计算机断层扫描显示纵隔多个淋巴结及肺中上部模糊的结节状病变。经支气管活检显示肺有肥大细胞浸润并形成肥大细胞肉芽肿。根据目前的文献，累及肺部的系统性肥大细胞疾病是一种非常罕见的病症。在用α-2a干扰素治疗6个月后，患者的病情尤其是呼吸困难有所改善，胸部计算机断层扫描显示病变也有所好转。
