CONGENITAL HYPOPLASTIC-ANEMIA IN 6 PATIENTS - UNUSUAL ASSOCIATION OF SHORT PROXIMAL PHALANGES WITH MILD ANEMIA

Six congenital hypoplastic anemia (CHA) patients from five families wh o have been followed from 2 months to 28 years are presented. Mild hhp oplastic anemia in a 13-year-old girl was associated with clinodactyly of the fifth finger on both hands, shortness in the proximal phalange s on all fingers, and syndactyly between the second and third toes and short fourth toe on the right foot. These abnormalities, except for c linodactyly, have not been reported previously in CHA. In one of the f ive families genetic transmission was thought to be autosomal-dominant since both the father and the son had the disease. Therapy with corti costeroids was initiated in all patients at the ages of 3.5 months to 13 years. Complete or near-complete recovery of anemia was obtained.