Article
Mutant elastase in pathogenesis of cyclic and severe congenital neutropenia.
Journal of pediatric hematology/oncology - 1 Dec 2002
Aprikyan Andrew A G, Liles W Conrad, Boxer Laurence A, Dale David C
Abstract excerpt
Severe neutropenia is characterized by maturation arrest of myeloid cells at the promyelocyte stage of hematopoiesis. We reported that accelerated apoptosis of bone marrow myeloid progenitor cells was observed in both cyclic (CN) and severe congenital neutropenia (SCN). Short and long-term cultures of bone marrow CD34+ cells revealed reduced production of multipotent progenitors in SCN. In contrast, production of...
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