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A 71-year-old man with a solitary red lesion on his left arm, which initially thought being a hematoma, was admitted for inpatient evaluation of anemia, which after a positive direct Coombs test was qualified as autoimmune hemolytic anemia. The patient also presented with hoarseness and biopsies from the swelled nasopharynx and an enlarged right tonsil were obtained. Due to uncertain diagnosis and the patient’s previous history of malignancy, (low grade lymphoproliferative lymphoma) skin and bone marrow biopsies were performed. Unfortunately, the patient’s status rapidly deteriorated as a result of septic shock. The obtained skin, nasopharynx, and tonsil biopsies became available after the patient died and showed marginal zone lymphoma with no evidence of lymphoproliferative neoplasm in the bone marrow. This case demonstrates the diagnostic complexity of nodal marginal zone lymphoma complicated by severe life-threatening autoimmune hemolytic anemia in an elderly patient with multimorbidity, which presents management challenges for health care providers.

A 71-Year-Old Man Presenting with Anemia and a Solitary Skin Lesion Associated with Primary Cutaneous Marginal Zone Lymphoma