Splenic marginal zone B-cell lymphoma regularly involves the spleen, often bone marrow, and more rarely the peripheral lymph nodes. The disease may be dominated by anemia caused by autoimmunity, splenomegaly and/or marrow repression.
Patients may live long with this disease.
The goal of treatment is to prolong survival with a palliative effect. A splenectomy is often indicated as the first treatment choice having a therapeutic effect as well as improving a possible hemolytic condition.
The effect of chemotherapy is poorer than for chronic lymphatic leukemia/lymphoma, but the effect of rituximab is good, given either alone or combined with chemotherapy (retrospective study).
The treatment principles are the same as for other B-cell lymphomas with low aggressiveness. This means that depending on age, comorbidity and disease aggressiveness, a choice can be made between rituximab monotherapy, chlorambucil, or C(H)OP and rituximab. Interferon-alpha-2-beta has been shown to provide good response in this group of lymphoma patients who are also hepatitis C positive.