Primary cutaneous marginal zone lymphoma with sequential development of nodal marginal zone lymphoma in a patient with selective immunoglobulin A deficiency.

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Multiple lymphoma subtypes occurring within one patient is rare in the context of B-cell lymphoma , and only few such cases have been reported in association with primary cutaneous marginal zone lymphoma (PCMZL ). We herein describe the case of a 43 -year -old patient who was diagnosed with PCMZL and subsequently developed a clonally unrelated nodal marginal zone lymphoma (MZL ). At the time of diagnosis of PCMZL , multiple skin lesions were present . The atypical lymphoid infiltrate showed monotypic expression of immunoglobulin light chain lambda and heavy chain (IgM ) on immunohistochemistry and an identical B-cell clone . No sign of systemic lymphoma was present in staging examinations . Complete remission was achieved utilizing rituximab . After a 3 -year clinical course of repetitive cutaneous relapses and remissions , the patient additionally developed nodal lymphoma involvement by MZL which , however , harbored an immunophenotype and a genetic clone distinct from the cutaneous lymphoma counterpart . Therefore , the rare occurrence of two different types of MZL with sequential evolution was diagnosed . In this uncommon case , we hypothesize that selective immunoglobulin A deficiency may play a promoting role for the metachronous development of the two MZL that occurred in our patient .