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Title: Polyglandular Autoimmune Syndrome in a Patient With Sickle Cell Anemia: Presented at AACE

"Polyglandular Autoimmune Syndrome in a Patient With Sickle Cell Anemia: Presented at AACE"

By Maggie Schwarz WASHINGTON, DC -- May 27, 2005 -- Physicians should not jump to the conclusion that diabetes in a patient with sickle cell anemia is caused by iron overload, say researchers. "If a patient with sickle cell anemia and iron overload comes down with diabetes, don't assume the diabetes is due to iron overload," warned Allison Elise Kerr, MBBS, a fellow in clinical endocrinology at Howard University in Washington, DC, United States. Dr. Kerr and colleagues described a 33-year-old Jamaican woman with sickle cell anemia who had had numerous blood transfusions and presently had iron overload. When she developed diabetes, it turned out to be due to type 3 polyglandular autoimmune syndrome rather than to iron overload. The investigators reported on this case here May 19[^th at the American Association of Clinical Endocrinologists (AACE) 14^th Annual Meeting and Clinical Congress.

Dr. Kerr said the course to take in a sickle cell patient who has come down with diabetes and has received multiple blood transfusions is to check for pancreatic antibodies. If they are high, the patient has confirmed type 1 diabetes. If pancreatic antibodies are absent, the diabetes was caused by iron overload.

"If the patient has one immune-mediated disease, check other endocrine organs," Dr. Kerr recommended. "Check for anti-thyroid antibodies, adrenal antibodies, and ovarian antibodies."

"Our patient had iron overload with alloantibodies against erythrocyte antigens, and type 3 polyglandular autoimmune syndrome (based on Neufield and Blizzard classification)," said Dr. Kerr.

Diabetes mellitus in the presence of iron overload is not an unusual finding in patients with sickle cell disease. The exact mechanism by which diabetes develops in the presence of iron overload has not been fully explained, and no published reports have suggested induction of antibody formation to mediate this effect of iron.

The investigators found no reports in the literature on the incidence or occurrence of sickle cell disease and polyglandular autoimmune syndrome, though some studies have reported on the presence of autoantibodies to rheumatologic organs with an increasing number of transfusions in patients with sickle cell disease.

The authors conclude that the numerous blood transfusions given to patients with sickle cell anemia, in addition to genetic and environmental factors, may increase this group's risk of developing autoimmune polyendocrinopathies and endocrine organ dysfunction, unrelated to iron overload.

[Presentation title: Polyglandular Autoimmune Syndrome in a Patient With Sickle Cell Anemia and Iron Overload. AACE: 914.]

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