Cederleuf Henrik, Bjerregård Pedersen Martin, Jerkeman Mats, Relander Thomas, d'Amore Francesco, Ellin Fredrik
Oncology and Pathology, Department of Clinical Sciences, Lund University, Lund, Sweden.
Department of Haematology, Aarhus University Hospital, Aarhus, Denmark.
Br J Haematol. 2017 Sep;178(5):739-746. doi: 10.1111/bjh.14740. Epub 2017 May 8.
Anaplastic large cell lymphomas (ALCLs) are rare CD30+ peripheral T-cell lymphomas (PTCLs) classified according to the expression of the anaplastic lymphoma kinase (ALK+) protein or not (ALK-). We have analysed the outcome and risk factors for survival in a population-based bi-national cohort of patients with systemic ALK+ ALCL. A total of 122 adult (≥18 years) patients diagnosed with ALK+ ALCL between 2000 and 2010 were identified from the Danish and Swedish lymphoma registries, representing 0·4% of all lymphomas. The median age of the cohort was 40 years (range 18-85). The 5-year overall survival and progression-free survival (PFS) was 78% and 64%, respectively. Age was strongly associated with outcome, and only bone marrow (BM) involvement was independently associated with poorer PFS in multivariate analysis (Hazard Ratio [HR] = 8·57, P < 0·001). Age stratification of the patients demonstrated an association between treatment with CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, prednisolone) and improved overall survival for patients aged 41-65 years, even when adjusted for risk factors (HR = 0·38, P = 0·047). Our results suggest that the addition of etoposide to CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) in the treatment for ALK+ ALCL seems reasonable in this age group.
间变性大细胞淋巴瘤（ALCL）是罕见的CD30+外周T细胞淋巴瘤（PTCL），根据间变性淋巴瘤激酶（ALK+）蛋白的表达与否分为ALK+和ALK-两类。我们分析了一个基于人群的两国队列中系统性ALK+ ALCL患者的生存结局和危险因素。从丹麦和瑞典淋巴瘤登记处识别出2000年至2010年间共122例诊断为ALK+ ALCL的成年（≥18岁）患者，占所有淋巴瘤的0.4%。该队列的中位年龄为40岁（范围18 - 85岁）。5年总生存率和无进展生存率（PFS）分别为78%和64%。年龄与结局密切相关，多因素分析中仅骨髓（BM）受累与较差的PFS独立相关（风险比[HR]=8.57，P<0.001）。对患者进行年龄分层显示，对于41 - 65岁的患者，即使校正了危险因素，CHOEP（环磷酰胺、多柔比星、长春新碱、依托泊苷、泼尼松龙）治疗与改善的总生存率相关（HR = 0.38，P = 0.047）。我们的结果表明，在该年龄组中，ALK+ ALCL的治疗在CHOP（环磷酰胺、多柔比星、长春新碱、泼尼松龙）基础上加用依托泊苷似乎是合理的。
