Marginal zone lymphoma of both spleen and kidney displaying transformation into large B-cell lymphoma

We report a case of simultaneous involvement of the spleen and the left kidney in a marginal zone lymphoma with a monotypic lymphoplasmacytic cell component, which transformed into a diffuse large B-cell lymphoma of the immunoblastic type. PCR showed that the small and large B-cell populations carried the same type of immunoglobulin heavy chain gene rearrangement. This type of rearrangement was detected in the spleen, the latero-aortic lymphadenopathy and the kidney demonstrating that it is the same lymphoma that affected both organs and the lymph nodes. Primary renal lymphoma is very rare and only a few cases of renal marginal zone lymphoma, MALT type, have been reported. Involvement of simultaneous multiple sites has been described in MALT type lymphoma, but splenic involvement secondary to renal MALT lymphoma seems to have never been observed. Nevertheless, in our case the huge size of the spleen associated with splenic hilar node involvement is consistent with primary splenic marginal zone lymphoma. The extension into latero-aortic lymph nodes of this lymphoma can explain secondary kidney involvement. The nodal Kaposi’s sarcoma observed in this patient of Mediterranean origin was probably coincidental.