Splenic marginal zone lymphoma

: Splenic marginal zone lymphoma (SMZL) with or without villous lymphocytes is a low-grade malignant lymphoma, which primarily involves the spleen, the bone marrow and the blood. Even though the prognosis of the disease is good and there is a long overall survival, about two thirds of the patients will require treatment and in 10% of the patients the lymphoma will transform into a high-grade malignant lymphoma. The treatment of SMZL remains empirical, since there is lack of large, prospective studies. Without such studies, the best treatment modality cannot be established.