Intermediate Grade (Moderately Aggressive) Lymphomas

Moderately aggressive lymphomas are subdivided into B-cell and T-cell subtypes.

B-cell Diffuse mixed (small and large) cell lymphoma
Mantle cell lymphoma
Follicle lymphoma (large cell)

T-cell T-cell chronic lymphocytic leukemia/prolymphocytic leukemia (T-CLL/PLL)
Adult T-cell leukemia/ lymphoma (ATL/L) [chronic]
Angiocentric lymphoma
Angioimmunoblastic lymphoma

Diffuse large cell lymphoma is the most common type of lymphoma, accounting for approximately one-third of all NHLs, regardless of grade. Patients typically report a history of a rapidly enlarging, symptomatic mass. Approximately two-thirds of all patients have widespread disease at diagnosis, and about 30% of patients also suffer from B symptoms. Prognosis tends to be strongly associated with the International Prognostic Index (IPI) score.

Mantle cell lymphoma is a tumor of the mantle cells that surround the germinal (reproducing) centers of the lymph nodes. Mantle cell lymphoma occurs Primarily in men (approximately 75%), and with an average patient age of 63, it rarely is seen in young patients. At the time of diagnosis, most patients show generalized lymph node disease, with or without enlargement of the spleen and liver. In addition, approximately two-thirds of all newly diagnosed patients have bone marrow involvement. Only about 20% of patients have Stage 1 or 2 disease at diagnosis.

The IPI score accurately predicts how most patients will do over time. Although this tumor is a small, B-cell lymphoma, it does not act like one—it is not slow growing and low-grade in character. Instead, the prognosis (predicted outcome) of mantle cell lymphoma is considerably poorer than what was initially expected. The reasons for this inconsistency are unclear, and so there is ongoing investigation into the biology of this disease. The clinical course of mantle cell lymphoma is intermediate between low-grade lymphomas and poorly responsive intermediate-grade lymphomas.

Adult T-cell leukemia/lymphoma (ATL/L) is seen less frequently in its chronic form. In chronic cases of ATL/L, the most noteworthy symptom is skin rash.

Angiocentric lymphoma is a type of T-cell lymphoma that is associated with Epstein Barr virus (EBV) infection. It includes cancers that previously were labeled "lethal midline granulomas," "nasal T-cell lymphoma," and "lymphatoid granulomatosis." Angiocentric lymphoma typically is characterized by a destructive nasal or midline facial tumor that is accompanied by invasive infiltrate, a substance that penetrates the spaces between tissues. The patient's palate (roof of mouth) may be destroyed by the tumor, and he or she may have prominent swelling of the eyes and face. Other sites of involvement outside the lymph nodes (extranodal) are the skin, lungs, and central nervous system. This tumor often is very resistant to therapy and has a poor prognosis. It is much more common in Asians than in people of European descent.

Angioimmunoblastic T-cell lymphoma (AILD) initially was thought to represent an abnormal reaction of the immune system. Now it is considered a variety of T-cell lymphoma. This malignancy usually occurs in adults. Many patients have generalized lymphatic disease (lymphadenopathy), hypergammaglobinemia (increased antibody proteins in the blood), and marked symptoms, including fever, weight loss, and skin rash. Although steroid therapy initially is beneficial in many patients, the disease usually progresses (e.g., to high-grade T-cell immunoblastic lymphoma).

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

Published: 15 Aug 1999

Last Modified: 02 Dec 2011