Brice P, Cazals D, Mounier N, Verola O, Neidhart-Berard A M, Remenieras L, Deconinck E, Doyen C, Hamelsand J, Molina T, Moulonguet I, Gisselbrecht C
Institut d' Hématologie, Hôpital Saint-Louis and Hotel-Dieu, Paris, France.
Leukemia. 1998 Feb;12(2):213-9. doi: 10.1038/sj.leu.2400911.
The objectives of this study were to evaluate the outcome after polychemotherapy for patients with primary cutaneous large-cell lymphomas (PCLL) and to validate the recently proposed immunohistologic classification of cutaneous lymphomas. Among 140 patients with positive skin biopsies included in the LNH87 protocol (for treatment of aggressive lymphomas), 49 patients met the criteria of PCLL. Characteristics were: sex ratio M/F, 2.3; age 18 to 83 years (median, 52), peripheral lymph nodes, n=22; diffuse disease, n=12; median tumor size, 4.5 cm; elevated lactate dehydrogenase, n=9; ECOG: 0/1, n=49. Histology was: follicular center B cell, n=23; B-lymphoblastic, n=1; anaplastic large-cell lymphoma, n=14 (T cell phenotype n=8); CD30- T cell lymphoma, n=11. All patients received polychemotherapy: under 70 years, ACVBP (three to four cycles and consolidation for 6 months) n=25; mBACOD (eight cycles) n=16; over 70 years, C(T)VP (six cycles) n=8. Radiation therapy was not included in the protocol. With a median follow-up of 5 years, 24/49 patients had relapsed, with 20 skin relapses. Event-free (EFS) and overall survival (OS) at 5 years were, respectively, 50 and 77%. Significant adverse prognostic factors were: histology (CD30- T cell lymphoma) and diffuse cutaneous disease (>10% of skin). The presence of nodal involvement was only significant for EFS. When compared to 140 non-cutaneous lymphoma patients included in the same trial and fully matched for the main clinical characteristics, OS was similar. In conclusion, PCLL behaves like other localized B or T cell extranodal lymphomas with the same prognostic factors (LDH, ECOG, age) except for CD30+ PCLL which have a very good prognosis.
本研究的目的是评估原发性皮肤大细胞淋巴瘤（PCLL）患者接受多药化疗后的结局，并验证最近提出的皮肤淋巴瘤免疫组织学分类。在LNH87方案（用于治疗侵袭性淋巴瘤）纳入的140例皮肤活检阳性患者中，49例符合PCLL标准。其特征为：男女比例为2.3；年龄18至83岁（中位数为52岁），有22例出现外周淋巴结受累；12例为弥漫性病变；肿瘤大小中位数为4.5 cm；9例乳酸脱氢酶升高；东部肿瘤协作组（ECOG）体能状态评分为0/1的有49例。组织学类型为：滤泡中心B细胞型23例；B淋巴母细胞型1例；间变性大细胞淋巴瘤14例（T细胞表型8例）；CD30 - T细胞淋巴瘤11例。所有患者均接受多药化疗：70岁以下患者，25例接受ACVBP方案（三至四个周期并巩固6个月）；16例接受mBACOD方案（八个周期）；70岁以上患者，8例接受C(T)VP方案（六个周期）。该方案未包括放射治疗。中位随访5年时，49例患者中有24例复发，其中20例为皮肤复发。5年无事件生存率（EFS）和总生存率（OS）分别为50%和77%。显著的不良预后因素为：组织学类型（CD30 - T细胞淋巴瘤）和弥漫性皮肤病变（皮肤受累>10%）。淋巴结受累仅对EFS有显著影响。与同一试验中纳入的140例非皮肤淋巴瘤患者相比，二者主要临床特征完全匹配，OS相似。总之，除CD30 + PCLL预后非常好外，PCLL的表现与其他具有相同预后因素（乳酸脱氢酶、ECOG体能状态评分、年龄）的局限性B或T细胞结外淋巴瘤相似。
