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.
Mandel
M
M
Department of Pediatrics B, Chaim Sheba Medical Center, Tel-Hashomer, Israel.
Etzioni
A
A
Theodor
R
R
Passwell
J H
JH
D000293:Adolescent; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications*; D002648:Child; D004700:Endocrine System Diseases / Q000097:blood / Q000150:complications*; D005260:Female; D006801:Humans; D008297:Male; D012010:Red-Cell Aplasia, Pure / Q000209:etiology*; D013577:Syndrome