2000 Associates' Presentations
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Chronic Autoimmune Hemolytic Anemia: Complications and Concomitant Diseases

Lisa Illig & Eliot Williams, University of Wisconsin Medical School, Madison, WI

Case: A 62 year old man with a 6 year history of idiopathic autoimmune hemolytic anemia (AIHA), was admitted with chest pain, shortness of breath and symptoms of an upper respiratory infection URI). Hemoglobin was 6 with 28% reticulocytes. Total bilirubin and LDH were elevated. Myocardial infarction was ruled out and he was given red cell transfusions with resolution of his symptoms. Symptoms were attributed to hemolysis triggered by URI. Prednisone was increased from 40 to 60 mg daily. Mycophenolate and azathioprine were continued. He presented 3 weeks later with daily fevers, chills and night sweats. Exam was unremarkable. Hemoglobin was 6.1 with 37% reticulocytes. Chest x-ray demonstrated a new diffuse nodular interstitial abnormality throughout both lung fields suspicious for miliary tuberculosis. Blood cultures were negative. No sputum was produced. PPD was anergic. CT of the chest, abdomen and pelvis revealed clear lung fields, but showed mediastinal, retroperitoneal, and left axillary lymphademopathy. Lymph node and bone marrow biopsies were performed. The lymph node showed mycobacterial lymphadenitis, with sheets of histiocytes full of acid-fast organisms. The marrow was 90% cellular with a monoclonal B-cell population consistent with chronic lymphocytic leukemia. Cultures of the lymph node and bone marrow grew Mycobacterium avium.

Discussion: Treatment of idiopathic AIHA begins with steroid therapy. If steroids cannot be tapered, splenectomy is performed. If hemolysis continues, other immunosuppressives are added, however the risk for opportunistic infection increases. Disseminated M. Avium is most commonly seen in patients with late-stage AIDS, but has been reported less frequently in patients with other causes of immunosuppression. Treatment in this case was complicated by underlying CLL and ongoing hymolysis.

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