Wells Jillian, Kosky Christopher A, Scolyer Richard A, Lee Stephen, Bye Peter T P, Young Graham A R, Davies Leo
Department of Dermatology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.
Australas J Dermatol. 2004 May;45(2):114-8. doi: 10.1111/j.1440-0960.2004.00058.x.
An unusual case of subcutaneous panniculitis-like T-cell lymphoma is presented involving multiple organ systems, which eventually culminated in rapid demise from the haemophagocytic syndrome, after an initial protracted course. A 44-year-old man presented in April 2001 with bronchiolitis obliterans organising pneumonia that initially responded well to corticosteroids. However, the condition relapsed on attempted prednisone withdrawal in January 2002 and the patient was noted to have developed truncal subcutaneous nodules. Initial skin biopsy revealed lobular panniculitis, with negative microbiological culture. In July 2002, mononeuritis multiplex was diagnosed after the patient presented with paresthesiae and was treated with pulse cyclophosphamide therapy. By November 2002 there was ulceration of the subcutaneous nodules. Repeat skin biopsy revealed subcutaneous panniculitis-like T-cell lymphoma. The clinical manifestations were supportive of an unifying diagnosis of malignancy involving pulmonary, cutaneous and nervous systems. Combination chemotherapy with fludarabine, mitoxantrone and dexamethasone was commenced. However, the patient deteriorated, with fevers, weight loss, pancytopenia and laboratory features consistent with the haemophagocytic syndrome. Despite maximal supportive therapy the patient succumbed to his disease.
本文报告了一例罕见的皮下脂膜炎样T细胞淋巴瘤病例，该病例累及多个器官系统，在经历了最初的漫长病程后，最终因噬血细胞综合征迅速死亡。一名44岁男性于2001年4月出现闭塞性细支气管炎伴机化性肺炎，最初对皮质类固醇治疗反应良好。然而，2002年1月尝试停用泼尼松后病情复发，发现该患者出现躯干皮下结节。最初的皮肤活检显示小叶性脂膜炎，微生物培养结果为阴性。2002年7月，患者出现感觉异常，诊断为多发性单神经炎，并接受了脉冲环磷酰胺治疗。到2002年11月，皮下结节出现溃疡。再次皮肤活检显示为皮下脂膜炎样T细胞淋巴瘤。临床表现支持对累及肺、皮肤和神经系统的恶性肿瘤进行统一诊断。开始使用氟达拉滨、米托蒽醌和地塞米松进行联合化疗。然而，患者病情恶化，出现发热、体重减轻、全血细胞减少以及与噬血细胞综合征相符的实验室检查特征。尽管给予了最大程度的支持治疗，患者仍死于该病。
