Treatment options depend on the stage and grade of the disease. The infrequent patients with early-stage disease may be treated with local radiation, with or without chemotherapy. In all types the deposits tend to be paratrabecular and to be composed mainly of the smaller cells. Peripheral blood involvement is most common in the small-cleaved type. Substantial involvement is usually a late phenomenon.
Spleen involvement is seen in about half the patients at laparotomy.
The small-cleaved and mixed types infiltrate and transform all the normal splenic follicles, forming innumerable tiny deposits. The large cell type forms large, irregular tumor masses. Usually the portal triads are affected. Most cases also react for CD10, which is also seen in lymphoblastic lymphoma but not other low grade lymphomas. No staining for CD5 is seen.