What Are the Types of Mature T-cell Lymphoma?

This content has been reviewed and approved by

James O. Armitage, MD
The Joe Shapiro Professor of Internal Medicine
Section of Oncology/Hematology
University of Nebraska Medical Center

There are many types of lymphomas of mature T cells. The majority of these lymphomas are similar to diffuse large B-cell lymphoma, but when the pathologist examines the slides, the tumor cells contain the T-lymphocyte traits. These lymphomas are slightly more likely to have wide spread involvement and have a poorer response to therapy than diffuse large B-cell lymphoma.

There are several specific subtypes of T-cell lymphoma that deserve mention. A very slow growing lymphoma in the skin is called mycosis fungoides--a specific type of cutaneous T-cell lymphoma displaying patchy, red skin inflammation, and tumors. This disorder is usually diagnosed by dermatologists, and the disease is frequently controlled for an extended period of time with local measures. However, it will eventually respond poorly to therapy, spread to lymph nodes and other organs, and be a very serious disease.

Anaplastic large T-cell lymphoma was only recently recognized. This lymphoma occurs at a younger median age than other T-cell lymphomas and is frequently seen in children. When this lymphoma is diagnosed, pathologists will look for a specific chromosomal abnormality and the presence of an abnormal protein called ALK. Patients who have the chromosomal abnormality and whose lymphomas overexpress the ALK protein probably have a better response to therapy than those who do not. However, with or without excessive production of the protein, this T-cell lymphoma responds to treatment better than others.

Angioimmunoblastic T-cell lymphoma is a specific type of T-cell lymphoma that tends to have wide spread lymphadenopathy; systemic symptoms, such as fevers; and abnormal laboratory results, such as an elevated level of proteins in the blood. This disease has a poorer outlook than the anaplastic large T-cell lymphomas.

There are a variety of rare T-cell lymphomas that have very characteristic findings. These include hepatosplenic T-cell lymphoma, with symptoms including an enlarged liver and spleen, bone marrow involvement, and low blood counts, but no lymphadenopathy. Enteropathy associated T-cell lymphoma involves the small bowel and is particularly likely to occur in patients with sprue (celiac disease).

Nasal/NK cell lymphoma is rare in the United States, but relatively frequent in southeast Asia and parts of Latin America. The disease usually involves a tumor affecting the nose and sinuses. Subcutaneous panniculitis-like T-cell lymphoma has tumors involving the skin and subcutaneous tissue . It has been particularly difficult to diagnose. Adult T-cell leukemia/lymphoma is associated with infection by a particular virus (for example, HTLV 1) and is rarely seen in most of the United States. These patients often have skin abnormalities or serious systemic symptoms. The disease is diagnosed by examining the blood, bone marrow, or lymph node biopsy. All of these rare T-cell lymphomas have a poor outlook.
.