Management of splenic marginal zone lymphoma

CR: complete remission; PR: partial remission.
* Splenectomy is an alternative for patients with localized symptoms due to splenomegaly. Splenectomy is not expected to benefit patients with disseminated nodal involvement outside of the splenic hilum or those with cytopenias due to extensive bone marrow involvement. Splenectomy is preferred for patients with suspected large cell transformation within the spleen. Asplenic patients are at risk for a fulminant sepsis syndrome, usually caused by encapsulated organisms. Whenever elective splenectomy is considered, patients should undergo appropriately timed preoperative immunization.
¶ The following autoimmune complications occur in approximately 20% of cases and require specific management: Autoimmune hemolytic anemia, immune thrombocytopenia, cold agglutinin disease, antiphospholipid antibodies, acquired von Willebrand disease, and acquired C1-esterase inhibitor deficiency.