Abstract
Three children with severe congenital neutropenia, 2 Kostmann type and 1 associated with dysgammaglobulinemia, were studied. Marrow aspirates were normocellular but lacked granulocytic cells beyond the myelocyte stage. In a methyl-cellulose culture system the numbers of granulocytic colony-forming units (CFU-C) were 246, 135, and 153/105 nucleated marrow cells respectively. These values were in the highest 10th percentile of a group of 47 control children (geometric mean of controls: 48 colonies/105 cells). The numbers of CFU-C in peripheral blood of 2 patients were 2.7 and 9.6/105 nucleated cells after ficoll-Hypaque separation (normal range 3.6 to 19.2). Differentiation in culture proceeded through all stages of granulopoiesis. Colony-stimulating activity (CSA) prepared from peripheral leukocytes of 2 patients demonstrated 88 and 106% of the activity of a normal CSA standard, in stimulating colony growth of several normal marrows. Urinary CSA levels were normal to increased. Varying amounts of patients' sera failed to inhibit colony formation by their own or control marrows, with or without CSA. No intrinsic granulocytic stem cell defect was demonstrated in these patients. A deficiency of CSA or defect in CSA-producing cells is unlikely. No inhibitor directed against granulocytic precursors or CSA was found. Therefore, the defect appears to be an abnormal hemopoietic microenvironment in vivo.
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