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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 investigate the clinical manifestations, pathological features, effective treatment and prognosis of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Methods The clinicopathological features, diagnosis, treatment and prognosis of 4 SPTCL patients confirmed by pathology were retrospectively reviewed and reviewed. Results In the clinical manifestations, the first symptom of 3 patients was subcutaneous nocturnal pressure-nodules, 1 case of cough as the first symptom, but also the subsequent skin damage; in addition to skin lesions, 1 patient developed hemophagocytic syndrome, 2 patients Invasion of bone marrow, 1 patient involved in the left adrenal. Pathology and immunophenotype of all patients were in line with SPTCL, subtypes of different sizes of atypical lymphocytes infiltration and arranged around a single fat cell lacy, showing mitotic, phagocytic phenomena and apoptotic bodies, And may be associated with vascular infiltration and granuloma-like changes; tumor cells express the immunophenotype of cytotoxic T cells. Treatment, 1 patient was 6 cycles of CHOP regimen achieved complete remission, but after 8 months of recurrence, the use of GELOX regimen to achieve partial remission after 5 cycles, the survival time was 39 months; the application of GELOX regimen in patients with untreated patients 2 cases, 1 case lost to follow-up, 1 case completely relieved, and the survival time was 7 months. One patient was treated with 5 cycles of chemotherapy to achieve hematopoietic stem cell transplantation after partial remission. The survival time was 28 months. In addition to a patient lost more than the other patients, the other survived. Conclusion SPTCL is a highly malignant invasive lymphoma. It progresses rapidly and mainly affects subcutaneous adipose tissue. Currently, there is no unified treatment regimen, and the curative effect of CHOP regimen is poor. Chemotherapy regimens with gemcitabine and asparaginase and timely hematopoietic stem cells Transplantation may be an effective treatment.