Aggressive and Very Aggressive (High Grade) Lymphomas

Small noncleaved cell lymphoma (SNCL) is a classification that, in general, has been replaced by the terms Burkitt's lymphoma and Burkitt-like (or non-Burkitt's) lymphoma. In children, most SNCLs are Burkitt's lymphomas, whereas most adult SNCLs are Burkitt-like lymphomas.

Burkitt's lymphoma tends to occur most often in children and in adults with compromised immune systems. There are three distinct forms of this disease: endemic, sporadic, and immunodeficient.

Endemic Burkitt's lymphoma is seen among the young of equatorial Africa. It frequently affects the jaws of children with developing molar teeth; experts suggest that growth factors may be responsible for the site-specific nature of this tumor.

Sporadic Burkitt's lymphoma often involves the abdomen, and it spreads to the bone marrow in about 20 percent of patients.

Immunodeficient Burkitt's lymphoma affects patients with abnormal immune systems (e.g., patients with HIV/AIDS).

When Burkitt's lymphoma involves the abdomen, it can cause symptoms such as abdominal pain, swelling, nausea, vomiting, and changes in bowel habits. It is a highly malignant lymphoma, but it is potentially curable with very aggressive therapy.

Patients with Burkitt-like lymphoma usually are treated similarly to those who have classic Burkitt's lymphoma.

Large cell immunoblastic lymphoma is considered to be a more aggressive form of diffuse large cell lymphoma, which is categorized as "intermediate" in nature. At diagnosis, this lymphoma is usually a systemic (whole-body) disease. Immunoblastic lymphoma is rapidly fatal if untreated, but frequently curable with intensive chemotherapy.

Lymphoblastic lymphoma often arises in young men, showing high rates around the 16-year and 40-year age groups. In women, there is a more even distribution of disease across all ages.

Lymphoblastic lymphoma has several clinical hallmarks, including the production of a mediastinal (middle chest) mass, involvement of the bone marrow and central nervous system (CNS), and "leukemia-like" characteristics similar to adult T-cell leukemia/ lymphoma (T-ALL). But lymph node disease (lymphadenopathy) prevails in lymphomblastic lymphoma, whereas peripheral blood changes prevail in T-ALL.

Lymphatic involvement usually occurs above the diaphragm, affecting the nodes of the neck, collarbone, or underarms. In the past, lymphoblastic lymphoma was associated with a poor prognosis; however, complete remission and long-term survival now can be accomplished with the use of intensive chemotherapy programs.

Publication Review By: Stanley J. Swierzewski, III, M.D.

Published: 14 Aug 1999

Last Modified: 28 Sep 2015