Yung A, Snow J, Jarrett P
Department of Dermatology, Waikato Hospital, Hamilton and Dermatology Unit, Greenlane Hospital, Auckland, New Zealand.
Australas J Dermatol. 2001 Aug;42(3):183-7. doi: 10.1046/j.1440-0960.2001.00511.x.
A 43-year-old Maori man presented with a 1 month history of malaise, weight loss, anorexia, arthralgia, recurrent fever and tender erythematous subcutaneous skin lesions. Histological examination of an incisional biopsy of a lesion revealed a lobular panniculitis with an inflammatory infiltrate of atypical lymphocytes and evidence of cytophagocytosis consistent with a diagnosis of subcutaneous T-cell lymphoma. The systemic symptoms and skin lesions resolved spontaneously within 3 weeks, only to recur 2 months later, requiring treatment with oral prednisolone. T-cell gene rearrangement studies demonstrated a monoclonal T-cell receptor (gamma-chain) gene rearrangement, further supporting the diagnosis of subcutaneous panniculitic T-cell lymphoma. Treatment with chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) led to remission of symptoms. Four months after completing chemotherapy, the patient remained asymptomatic with a few indurated subcutaneous plaques on the chest. Biopsy of these areas revealed lobular panniculitis, lymphocytic infiltrate without cytological atypia, abundant lipophages and fibrosis and sclerosis consistent with a healing response. He remains well 24 months following chemotherapy.
一名43岁的毛利男子出现了为期1个月的不适、体重减轻、厌食、关节痛、反复发热以及压痛性红斑皮下皮肤病变。对一处病变进行切开活检的组织学检查显示为小叶性脂膜炎,伴有非典型淋巴细胞的炎性浸润以及噬血细胞现象,符合皮下T细胞淋巴瘤的诊断。全身症状和皮肤病变在3周内自行消退,但2个月后复发,需要口服泼尼松龙进行治疗。T细胞基因重排研究显示单克隆T细胞受体(γ链)基因重排,进一步支持皮下脂膜炎性T细胞淋巴瘤的诊断。化疗(环磷酰胺、阿霉素、长春新碱和泼尼松)治疗使症状缓解。完成化疗4个月后,患者无症状,胸部有一些硬结性皮下斑块。对这些部位进行活检显示为小叶性脂膜炎,淋巴细胞浸润但无细胞学异型性,有大量脂肪吞噬细胞以及符合愈合反应的纤维化和硬化。化疗后24个月他仍状况良好。