RADIAL APLASIA AND AMEGAKARYOCYTIC THROMBOCYTOPENIA (TAR SYNDROME) AMONG NIGERIAN CHILDREN

Five patients with radial aplasia studied during a 10 yr period, had the amegakaryocytic thrombocytopenia syndrome. Together, they are termed the TAR syndrome. Two of the 5 patients were siblings from a polygamous family; 3 patients had relative hypogammaglobulinemia, and 1 of these patients had late onset of congenital varicella. The findings in these 5 cases led to further understanding of the genetic mechanisms of the TAR syndrome. The high incidence of infection associated with the syndrome could be partially explained by hypogammaglobulinemia.