Complex alpha-thalassemia-like syndrome: a cause of neonatal normoblastemia.

A previously unrecognized hypochromic anemia associated with marked normoblastemia during the newborn period is reported. One male and two female siblings and a first cousin had a hypochromic anemia and marked normoblastemia (300 to 900 normoblast index per 100 white blood cells) at birth. Globin chain synthesis studies on peripheral blood of the proband at birth indicated the presence of alpha-thalassemia trait with possible reduced gamma chain synthesis. Studies of globin chain synthesis on the father, two older affected siblings of the proband, and the proband at 1.5 years of age revealed alpha-thalassemia trait. The data suggest this complex alpha-thalassemia-like condition as a new syndrome associated with marked neonatal normoblastemia.

Lack of erythropoietic inhibitory effect of serum from patients withe congenital pure red cell aplasia.

The serum from five children with congenital pure red cell aplasia demonstrated no erythropoietic inhibitory effect either in vivo in animals or in vitro. The significance of immunosuppressive therapy in this disease is discussed.