皮下脂膜炎样T细胞淋巴瘤4例并文献复习

Four Cases of Subcutaneous Panniculitis-like T Cell Lymphoma with Literature Review

目的探讨皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床表现、病理特点、有效的治疗方案及预后。方法回顾性分析经病理证实的4例SPTCL患者的临床病理特征、诊断治疗及预后,并进行文献复习。结果临床表现上,3例患者首发症状均为皮下无压痛性结节,1例以咳嗽为首发症状,但后续亦出现皮肤损害;除皮肤损害外,1例患者出现噬血细胞综合征,2例侵犯骨髓,1例患者左侧肾上腺受累。所有患者的病理学和免疫表型均符合SPTCL,皮下脂肪组织内大小不一的非典型性淋巴细胞浸润并围绕单个脂肪细胞呈花边样排列,可见核分裂、组织细胞吞噬现象及凋亡小体,并可伴有血管浸润和肉芽肿样改变;肿瘤细胞表达细胞毒T细胞的免疫表型。治疗上,1例患者予以6周期CHOP方案化疗后达到完全缓解,但8个月后出现复发,使用GELOX方案治疗5周期后达到部分缓解,生存时间为39个月;应用GELOX方案治疗初治患者2例,1例失访,1例完全缓解,生存时间7月;1例患者5周期化疗达到部分缓解后行造血干细胞移植,生存时间为28个月。以上患者除1例失访外,其他均存活。结论 SPTCL是一种高度恶性的侵袭性淋巴瘤,进展快,主要累及皮下脂肪组织,目前尚无统一治疗方案,CHOP方案疗效欠佳,含吉西他滨及门冬酰胺酶的化疗方案和及时的造血干细胞移植可能成为有效的治疗手段。

Objective To explore the clinical manifestation, pathological features, effective treatment regimen and prognosis of subcutaneous panniculitis like T cell lymphoma （SPTCL）. Methods The clinicopathologic features, diagnosis treatment and prognosis of 4 SPTCL patients which confirmed by pathology were analyzed retrospectively. Results Clinically, subcutaneous nodules with no tenderness appeared firstly in three patients, and cough in one patient, but all followed by skin changes. Except for skin leisions, hemophagocytic syndrome developed in one patient, bone marrow was involved in two cases and adrenal gland in one. The pathology and immunophenotype results of all patients were accorded with subcutaneous panniculitis like T cell lymphoma. Atypical lymphocytes infiltrated and rounded the lipocytes like rosettes in subcutaneus adipose tissue. Mitosis, phagothrocytic phenomena and apoptotic body were also seen, accompanied by vascular infiltration and granulomatoid reaction. Tumor ceils showed immunophenotype of cytotoxic T lymphocytes. One patient received 6 cycles of CHOP chemotherapy and obtained complete response. However, he relapsed 8 months later, then he underwent 5 cycles of GELOX chemotherapy and partial response was achieved, with a survival time of 39 months. GELOX chemotherapy was used as initial therapy for two patients. One patient was lost for follow up after one cycle and the other achieved complete response, with a survival time of 7 months. One patient underwent autologous hematopoietic stem cell transplantation after 5 cycles of chemotherapy and partial response was achieved, and he survives for 28 months up to now. All the patients are alive except one lost follow. Conclusion Subcutaneous panniculitis like T cell lymphoma is a highly aggressive lymphoma with rapid progression. It mainly involves subcutaneus adipose tissues. At present, standard treatment has not been established yet, and the efficacy of CHOP regimen is unsatisfactory. Gemcitabine and L-Asparaginasum based chemotherapy and hematopoietic stem cell transplantation may become effective treatments.