Rojnuckarin Ponlapat, Nakorn Thanyaphong Na, Assanasen Thamathorn, Wannakrairot Pongsak, Intragumtornchai Tanin
Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailland.
Leuk Lymphoma. 2007 Mar;48(3):560-3. doi: 10.1080/10428190601078456.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of hematologic malignancy characterized by lesions in subcutaneous fat associated with systemic symptoms. The standard treatment of the disease, currently, is not established, but CHOP or CHOP-like regimens are usually given. We report, herein, 4 cases of SPTCL diagnosed by histopathology and immunohistochemistry who were refractory to CHOP and/or ESHAP and/or fludarabine-based regimen, but showed rapid improvement within weeks after oral cyclosporin 4 mg/kg/day. Three sustained complete remission for the durations of 8 - 9 months off-treatments. T-cell receptor gene rearrangement revealed polyclonality in 3 cases and monoclonality in 1 case. Our data suggest the benefit of incorporating cyclosporin into the treatment regimen for SPTCL.
皮下脂膜炎样T细胞淋巴瘤（SPTCL）是一种罕见的血液系统恶性肿瘤，其特征为皮下脂肪出现病变并伴有全身症状。目前该病的标准治疗方案尚未确立，但通常采用CHOP或类似CHOP的方案。在此，我们报告4例经组织病理学和免疫组织化学诊断为SPTCL的患者，他们对CHOP和/或ESHAP和/或基于氟达拉滨的方案耐药，但在口服环孢素4mg/kg/天数周后病情迅速改善。3例患者在停止治疗后持续完全缓解8 - 9个月。T细胞受体基因重排显示3例为多克隆性，1例为单克隆性。我们的数据表明将环孢素纳入SPTCL治疗方案有益。
