Reticular dysgenesis: report of two brothers.
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).
D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000473:pathology; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000235:genetics*; D007970:Leukopenia / Q000235:genetics*; D008221:Lymphoid Tissue / Q000473:pathology; D008297:Male