Defective Myelopoiesis in Congenital Neutropenia

Abstract

Two patients with congenital neutropenia were found to have abnormalities of the immature myeloid cells. These cells were incapable of complete differentiation to a mature stage and also had a substantial reduction in their capacity for cell proliferation. The defect in one patient appeared to be intrinsic to an abnormal cell line, but in the other bone-marrow environmental factors may have contributed to the defect in maturation and proliferation. The surprisingly benign clinical courses may be explained by compensatory defense mechanisms.