Originally published as JCO Early Release 10.1200/JCO.2008.21.5020 on July 27 2009

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Anaplastic Lymphoma Kinase–Positive Diffuse Large B-Cell Lymphoma: A Rare Clinicopathologic Entity With Poor Prognosis

Camille Laurent, Catherine Do, Randy D. Gascoyne, Laurence Lamant, Loïc Ysebaert, Guy Laurent, Georges Delsol, Pierre Brousset

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.

Corresponding author: Pierre Brousset, MD, PhD, Laboratoire d'Anatomie Pathologique, Centre Hospitalier Universitaire Purpan, place Baylac, Toulouse, F-31300 France; e-mail: brousset.p{at}chu-toulouse.fr.

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.

G.D. and P.B. share senior authorship of this article.

Authors' disclosures of potential conflicts of interest and author contributions are found at the end of this article.

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