[Congenital aplastic anemias]

Abstract

Congenital (constitutional) aplastic anemias in children are relatively rare compared to other diseases but as their outcome is nearly always fatal they are given full attention in pediatric-hematological literature. Although in many patients the number of white cells and thrombocytes as well as of red cells is diminished even in the first phase of the disease (or later in the course of the disease) so that these diseases are really pancytopenias, the term most frequently used to describe these clinical entities is aplastic anemia. The clinical and hematological characteristics of some aplastic anemias (Fanconi's anemia, Blackfan-Diamond's anemia, congenital dyserythropoietic anemia--type I, II, III and IV, etc.) are described. After considering the possible pathomechanisms which bring about aplastic anemia (pancytopenia), information is given about the method of treatment and prognostic prospects with reference to cases treated by the authors.