Hereditary erythroblastic multinuclearity associated with a positive acidified-serum test: a type of congenital dyserythropoietic anaemia

Summary. In five patients (including two sisters) an unusual anaemia was characterized by erythroblastic multinuclearity, ineffective erythropoiesis and a positive acidified-serum test. Unlike PNH, the sugar-water test was always negative in these patients. The lysis of their cells by acidified normal sera indicated their abnormal sensitivity to an agglutinating and complement-binding antibody present in some normal subjects. The patients cells gave high agglutination scores with anti-i, and were unusually susceptible to lysis by anti-i and anti-I. The disorder is apparently inherited as an autosomal recessive character.