Subsequently, peripheral lymph nodes and extra-lymph node organs, liver for instance, can be infiltrated with the disease progressing.[2] Meanwhile, the patients with SMZL frequently combine with autoimmune disease such as autoimmune hemolytic anemia, Sjogren syndrome and immune thrombocytopenia.[3] In spite of its indolence, approximately 10% of individuals with SMZL probably convert into aggressive diffuse large B-cell lymphoma.[4] Furthermore, about one third of patients exist plasmocytic differentiation and secrete monoclonal paraproteins including IgM predominantly. This evidence concerns the gene CD40LG and splenic marginal zone lymphoma.