Julie M. Vose, MD, MBA

Articles by Julie M. Vose, MD, MBA

Querfeld et al have written a comprehensive evaluation of primary cutaneous and systemic anaplastic large cell lymphoma (ALCL). The important similarities between them are outlined in the article, for example tumor cells expressing the CD30 antigen for both subtypes. In addition, important differences such as the wide spectrum of CD30+ lymphoproliferative disorders of the skin and the large differences in outcomes of the primary cutaneous vs systemic types of ALCL are discussed. Other important differences include expression of the anaplastic lymphoma kinase (ALK) protein. The ALK protein is activated most frequently through the nonrandom t(2;5) chromosome translocation, resulting in fusion of the nucleophosim (NPM) gene located at 5q35 to 2p23 and encoding the receptor tyrosine kinase, ALK. Most patients with primary cutaneous ALCL are ALK-negative, yet their 5-year disease-free survival rate is > 90%. Systemic ALCL is divided into ALK-positive and ALK-negative cases. Patients with systemic ALCL that is ALK-positive have a much higher 5-year survival rate of 70%, compared with a rate of 49% for those with systemic ALCL that is ALK-negative.[1]

Burkitt lymphoma (BL) is a unique B-cell lymphoma characterized by a high proliferation rate and cytogenetic changes related to c-myc proto-oncogene overexpression. Burkitt lymphoma is a highly aggressive B-cell lymphoma that is most frequently seen in children and young adults in endemic areas.

The most common indolent lymphoma, follicular lymphoma comprises 35% of adult non-Hodgkin’s lymphoma (NHL) in the United States and 22% worldwide. Features associated with adverse outcome include age, male gender, disease stage, and performance status, with the International Prognostic Index being the most widely used risk classification system. Long-term disease-free survival is possible in select patient subgroups after treatment, but very late relapses suggest that quiescent lymphoma cells might be harbored for long periods of time. Radiation therapy is the mainstay of treatment for limited-stage follicular lymphoma, but there is some experience with chemotherapy and combined chemoradiation. When to initiate treatment in patients with advanced disease is controversial, but options include various combined chemotherapy regimens, monoclonal antibodies, radiolabeled antibodies, and bone marrow or stem cell transplantation. Future directions in the treatment of follicular lymphoma include vaccines, antisense therapy, and proteasome inhibitors.