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Splenic Marginal Zone Lymphoma (SMZL) - Treatment & Prognosis: SMZL is an indolent, low-grade lymphoma with a 5-yr survival rate ranging between 65% to 75%. Factors associated with adverse outcome include TP53 mutation or overexpression, del 7q, and the absence of somatic mutation in IgVH genes (usually present in about 50% of cases).
Treatment options include splenectomy, fludarabine, chlorambucil, and, more recently, rituximab. Hepatitis C-associated cases may go into remission with anti-viral therapy.
Transformation to diffuse large B-cell lymphoma (DLBCL) occurs in 13-15% of cases of SMZL. Cases with 7q deletion appear to be at a greater risk of transformation. In contrast, the transformation rates with CLL and follicular lymphomas are 1-10% and 25%-60% respectively.
Treatment options include splenectomy, fludarabine, chlorambucil, and, more recently, rituximab. Hepatitis C-associated cases may go into remission with anti-viral therapy.
Transformation to diffuse large B-cell lymphoma (DLBCL) occurs in 13-15% of cases of SMZL. Cases with 7q deletion appear to be at a greater risk of transformation. In contrast, the transformation rates with CLL and follicular lymphomas are 1-10% and 25%-60% respectively.