Laurent Camille, Do Catherine, Gascoyne Randy D, Lamant Laurence, Ysebaert Loïc, Laurent Guy, Delsol Georges, Brousset Pierre
L'Institut National de la Santé et de la Recherche Médicale, U.563, Centre de Physiopathologie de Toulouse-Purpan, Toulouse, France.
J Clin Oncol. 2009 Sep 1;27(25):4211-6. doi: 10.1200/JCO.2008.21.5020. Epub 2009 Jul 27.
Anaplastic lymphoma kinase (ALK) -positive diffuse large B-cell lymphoma (DLBCL) is a rare variant of DLBCL that has been described only in small case reports. To shed more light on the clinical and pathologic features and outcome of these tumors, we reviewed data from 38 patients.
We retrospectively analyzed 38 patients with ALK-positive DLBCL treated with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) or CHOP-like regimens from different institutions to better define the presenting features, clinical course, and response to treatment.
The histologic findings in all patients were similar. All patients expressed ALK fusion proteins, but virtually all were CD30 and CD20 negative. The median age was 43 years with a 5:1 ratio of males to females. Most patients (60%) followed an aggressive clinical course with advanced stage at diagnosis, frequent marrow infiltration, and poor outcome. Overall survival was 20.3 months (95% CI, 12.2 to 42.6 months). Of note, the median survival was only 12.2 months (95% CI, 9.1 to 32.5 months) in patients with advanced-stage disease.
ALK-positive DLBCLs display clinicopathologic features that distinguish them from common DLBCL. Conventional therapy, as used for typical DLBCL, is of limited efficacy. Recognition of this new entity and the characteristic lack of CD20 expression are paramount. Novel front-line intensive chemotherapy regimens should be evaluated in this group of patients.
间变性淋巴瘤激酶（ALK）阳性弥漫性大B细胞淋巴瘤（DLBCL）是DLBCL的一种罕见变异型，仅在少数病例报告中有所描述。为了更深入了解这些肿瘤的临床和病理特征及预后，我们回顾了38例患者的数据。
我们回顾性分析了38例接受环磷酰胺、阿霉素、长春新碱、泼尼松（CHOP）或类似CHOP方案治疗的ALK阳性DLBCL患者，这些患者来自不同机构，以更好地明确其临床表现、临床病程及对治疗的反应。
所有患者的组织学表现相似。所有患者均表达ALK融合蛋白，但几乎所有患者CD30和CD20均为阴性。中位年龄为43岁，男女比例为5:1。大多数患者（60%）临床病程呈侵袭性，诊断时处于晚期，骨髓浸润频繁，预后较差。总生存期为20.3个月（95%CI，12.2至42.6个月）。值得注意的是，晚期疾病患者的中位生存期仅为12.2个月（95%CI，9.1至32.5个月）。
ALK阳性DLBCL具有与常见DLBCL不同的临床病理特征。用于典型DLBCL的传统治疗疗效有限。认识到这一新实体以及特征性的CD20表达缺失至关重要。应在这组患者中评估新型一线强化化疗方案。
