ALK-positive anaplastic large cell lymphoma in adults

Abstract

ALK-positive anaplastic large cell lymphoma (ALCL) represents approximately 6–7% of the mature T-cell lymphomas. This subtype contains a translocation between the ALK gene on chromosome 2 and one of several other genes that together form an oncogene. The most frequent translocation is t(2;5) which combines ALK with NPM1. This lymphoma has a median age of 34 years, is more common in males, and is in advanced stage at the time of diagnosis in most patients. ALK-positive ALCL is the most curable of the peripheral T-cell lymphomas. The CHOP regimen has been most frequently used, but results are improved with the substitution of brentuximab vedotin for vincristine (BV-CHP) and the addition of etoposide (CHOEP), with BV-CHP being favored. Salvage therapies include allogeneic or autologous bone marrow transplantation, BV, if not used as part of the primary therapy, and ALK inhibitors. The latter are very active and likely to be incorporated into the primary therapy.

Podcast on Emerging Treatment Options for Pediatric Patients with ALK-Positive Anaplastic Large Cell Lymphoma and Inflammatory Myofibroblastic Tumors

“…Between 65% and 80% of pediatric patients are cured using this approach [ 9 ]. While adult patients with ALCL tend to receive CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) [ 10 ], pediatric patients usually receive six cycles of multiagent chemotherapy similar to CHOP, as shown in the ALCL99 and the ANHL12P1 studies [ 11 , 12 ]. These patients undergo computed tomography/positron emission tomography (CT/PET) imaging 1 month after completion of therapy to assess response.…”