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Small noncleaved cell lymphoma (SNCL) is a classification that, in general, has been replaced by the terms Burkitt's lymphoma and Burkitt-like (or non-Burkitt's) lymphoma.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% 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.
