Reticular dysgenesis: report of two brothers.

Abstract

We describe two brothers with marked leucopenia, lymphopenia, no immunologicl response to infections (no Ig production, negative PHA response and very low number of T and B lymphocytes in peripheral blood) and hypocellular marrow. They died at 12 and 8 days of life with infection (E. coli and Klebsiella, respectively).