Primary cutaneous marginal zone B-cell lymphoma (PCMZBCL) is an entity with an indolent behavior, which clinically appears as erythematous papules, nodules, or plaques, solitary or multiple, on the trunk or upper extremities. It has been associated with autoimmune diseases and infections. We present the case of a 77-year-old woman with an atypical PCMZBCL with extracutaneous spread and associated autoimmune hemolytic anemia.
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