Romero L S, Goltz R W, Nagi C, Shin S S, Ho A D
Division of Dermatology, University of California, San Diego, School of Medicine, USA.
J Am Acad Dermatol. 1996 May;34(5 Pt 2):904-10. doi: 10.1016/s0190-9622(96)90077-8.
We report a case of subcutaneous T-cell lymphoma that presented as recurrent subcutaneous nodules, pancytopenia, and fever. Histopathologic examination revealed a dense infiltrate of markedly atypical lymphoid cells localized to the panniculus. These cells were identified as T cells by immunohistochemistry. There was associated karyorrhexis and fat necrosis. Hemophagocytosis was present both in the panniculus and in the bone marrow, with no tumor evident outside the subcutaneous tissue. Despite chemotherapy in conjunction with an autologous bone marrow transplant, the patient died after metastases, including explosive leukemic transformation, developed. Review of the literature shows subcutaneous T-cell lymphoma to be a rare peripheral T-cell lymphoma, often mistaken initially as a benign panniculitis, that manifests an aggressive, fulminant presentation in approximately one half of the patients; the remainder transform into a high-grade malignancy after months to years. The hemophagocytic syndrome, though to be a reactive T-cell process mediated by cytokines, is a frequent complication of this lymphoma and is responsible for its poor prognosis. Our patient uniquely demonstrated fatal leukemic transformation.
我们报告了一例皮下T细胞淋巴瘤病例，该病例表现为复发性皮下结节、全血细胞减少和发热。组织病理学检查显示，在皮下脂肪层有密集浸润的明显非典型淋巴细胞。通过免疫组织化学鉴定这些细胞为T细胞。存在核碎裂和脂肪坏死。皮下脂肪层和骨髓中均有噬血细胞现象，皮下组织外未发现明显肿瘤。尽管进行了化疗并联合自体骨髓移植，但患者在出现转移（包括爆发性白血病转化）后死亡。文献回顾显示，皮下T细胞淋巴瘤是一种罕见的外周T细胞淋巴瘤，最初常被误诊为良性脂膜炎，约一半患者表现为侵袭性、暴发性病程；其余患者在数月至数年后转变为高级别恶性肿瘤。噬血细胞综合征虽然被认为是由细胞因子介导的反应性T细胞过程，但却是这种淋巴瘤的常见并发症，也是其预后不良的原因。我们的患者独特地表现出致命的白血病转化。
