Affiliation:
1. From L'Institut National de la Santé et de la Recherche Médicale, U.563, Centre de Physiopathologie de Toulouse-Purpan; Université Paul-Sabatier; Service d'Hématologie, Centre Hospitalier Universitaire Purpan, Toulouse, France; Laboratoire d'Anatomie Pathologique, and British Columbia Cancer Agency, Vancouver, British Columbia, Canada.
Abstract
Purpose 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. Patients and Methods 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. Results 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. Conclusion 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.
Publisher
American Society of Clinical Oncology (ASCO)
Reference28 articles.
1. G Delsol, E Ralfkiaer, H Stein , etal: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues: World Health Organization Classification of tumours 230– 235,2001 Lyon, France IARC Press
2. BN Nathwani, HN, D Weisenburger , etal: Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. World Health Organization Classification of Tumours 162– 167,2001 Lyon, France IARC Press
3. ALK-positive diffuse large B-cell lymphoma is associated with Clathrin-ALK rearrangements: report of 6 cases
4. A New Subtype of Large B-Cell Lymphoma Expressing the ALK Kinase and Lacking the 2; 5 Translocation
Cited by
137 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献