Wollina Uwe, Langner Dana, Hansel Gesina, Haroske Gunter
Department of Dermatology and Allergology, Insitute of Pathology "Georg Schmorl", Academic Teaching Hospital Dresden-Friedrichstadt, Dresden, Germany.
Medicine (Baltimore). 2016 Oct;95(43):e4796. doi: 10.1097/MD.0000000000004796.
Cutaneous composite lymphomas are very rare. Their treatment depends upon the different contributing lymphoma entities. Peripheral T-cell lymphoma, not otherwise specified, (PTCL-NOS) represents an aggressive lymphoma subtype. Follicular cutaneous B-cell lymphoma (FCBCL) runs an indolent course. Treatment with pegylated liposomal encapsulated doxorubicin (PLE-DOXO) has yet not been reported in this entity.
A 73-year-old male patient presented with 3 rapidly growing, painful nodules on his left leg. He was diagnosed as composite cutaneous lymphoma consisting of PTCL-NOS and FCBCL. All lesions had been surgically removed. Staging was unremarkable. After 4 months a relapse occurred with involvement of inguinal lymph nodes and systemic treatment with PEL-DOXO 20 mg/ m every 3 weeks was initiated. After 6 cycles PLE-DOXO, which were well tolerated without grade 3 or 4 toxicities, a mixed response was obtained with complete remission of cutaneous lesions.Lymph nodes were treated by radiotherapy. A second relapse occurred after 8 months and various polychemotherapy regimens were applied without remission. The overall survival was 28 months.
PEL-DOXO is a possible initial systemic treatment in case of PCTL-NOS. Whether polychemotherapy offers an advantage for survival remains questionable but further investigations are needed.
皮肤复合型淋巴瘤非常罕见。其治疗取决于不同的淋巴瘤构成成分。外周T细胞淋巴瘤，非特指型（PTCL-NOS）是一种侵袭性淋巴瘤亚型。滤泡性皮肤B细胞淋巴瘤（FCBCL）病程呈惰性。聚乙二醇化脂质体包裹阿霉素（PLE-DOXO）治疗该疾病尚未见报道。
一名73岁男性患者左腿出现3个迅速增大的疼痛性结节。他被诊断为包含PTCL-NOS和FCBCL的复合型皮肤淋巴瘤。所有病灶均已手术切除。分期检查无异常。4个月后复发，累及腹股沟淋巴结，开始每3周用20mg/m的PEL-DOXO进行全身治疗。6个周期的PLE-DOXO治疗后，耐受性良好，无3级或4级毒性反应，皮肤病变完全缓解，获得混合反应。淋巴结接受放疗。8个月后再次复发，应用多种联合化疗方案均未缓解。总生存期为28个月。
对于PTCL-NOS，PEL-DOXO是一种可能的初始全身治疗方法。联合化疗是否对生存有优势仍存在疑问，但需要进一步研究。
