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Case Reports
. 1981 Feb 13;245(6):602-4.

Familial hypocomplementemic hemolytic uremic syndrome with HLA-A3,B7 haplotype

L Carreras, R Romero, C Requesens, A J Oliver, M Carrera, M Clavo, J Alsina
  • PMID: 7452889
Case Reports

Familial hypocomplementemic hemolytic uremic syndrome with HLA-A3,B7 haplotype

L Carreras et al. JAMA. .

Abstract

We describe a familial, relapsing type of hemolytic uremic syndrome (HUS) that has a possible genetic origin. Three members of a family, aged between 15 months and 34 years, evidenced HUS. Histological examination disclosed thrombotic microangiopathy. Recurrences, persistent hypocomplementemia, and the HLA haplotype A3,B7 were a common factor. We studied 17 members of this family and found a low C3 level from activation of the alternative pathway in the haplotype A3,B7 carriers. These three patients with HUS probably had a congenital and hereditary condition in which immunologic factors played a role in its pathogenesis.

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