Selective IgA deficiency with unusual features: Development of common variable immunodeficiency, Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura


SANAL Ö., ERSOY F., METİN A., TEZCAN Í., BERKEL A. Í., YEL L.

Pediatrics International, cilt.37, sa.4, ss.526-529, 1995 (Scopus)

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 37 Sayı: 4
  • Basım Tarihi: 1995
  • Doi Numarası: 10.1111/j.1442-200x.1995.tb03369.x
  • Dergi Adı: Pediatrics International
  • Derginin Tarandığı İndeksler: Scopus
  • Sayfa Sayıları: ss.526-529
  • Anahtar Kelimeler: autoimmune hemolytic anemia, common variable immunodeficiency, heterozygous C4 deficiency, immune thrombocytopenic purpura, selective IgA deficiency, Sjögren's syndrome
  • Sağlık Bilimleri Üniversitesi Adresli: Evet

Özet

We report on a girl with selective IgA deficiency and persistently low complement component 4 (C4) levels compatible with heterozygous C4 deficiency. Deterioration of her serum immunoglobulin levels and transition to common variable immunodeficiency were observed within a 5 year follow‐up. She also developed Sjögren's syndrome, autoimmune hemolytic anemia and immune thrombocytopenic purpura While these abnormalities have been described before in various combinations, to our knowledge, they have not been reported in a single individual. 1995 Japan Pediatric Society