Pure red cell hypoplasia associated with polyglandular autoimmune syndrome type I

The polyglandular autoimmune syndrome Type I is characterized by hypoparathyroidism, adrenal insufficiency, and mucocutaneous candidiasis, and may be associated with other autoimmune-mediated diseases, including pernicious anemia, chronic active hepatitis and vitiligo. We report two patients aged 7 and 15 years in whom pure red cell hypoplasia was a prominent feature of the polyglandular Type I syndrome. Hematological remission was obtained with corticosteroid treatment in one patient, and with gamma-globulin therapy in the other. These findings indicate that pure red cell hypoplasia is one of the autoimmune manifestations that may be associated with this syndrome.