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Hemopoietic marrow function in chronic neutropenia of blacks: Cure of aplastic anemia by allogeneic marrow transplantation from a neutropenic sibling donor
Author(s) -
Ash Robert C.,
Mendelsohn Larry A.,
Marshall M. Ernest
Publication year - 1986
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830220212
Subject(s) - neutropenia , aplastic anemia , haematopoiesis , medicine , sibling , immunology , bone marrow , transplantation , granulocyte , progenitor cell , anemia , granulocyte colony stimulating factor , stem cell , chemotherapy , biology , genetics , psychology , developmental psychology
A black patient with severe aplastic anemia is described who underwent successful bone marrow transplantation from a sibling with chronic neutropenia. During an evaluation to identify a suitable donor, it was found that the majority of famiily members tested had neutropenia, with no familial history of significant infections or related hospitalizations. In vitro hemopoietic culture studies of marrow from the patient's HLA‐MLC‐matched siblings showed normal numbers of pluripotential and committed hemopoietic progenitors; in vitro hemopoietic colony formation from the patient was markedly subnormal, consistent with the clinical picture of severe aplastic anemia. Following appropriate conditioning therapy, marrow transplanted from one of these neutropenic sibs produced full hematopoietic reconstitution. Posttransplant marrow culture studies of the patient showed restoration of a normal pattern of in vitro hemopoiesis. The in vitro culture studies and clinical experience in this patient support the concept that chronic neutropenia of blacks is not primarily a marrow progenitor cell disorder but, more likely, a manifestation of a genetically determined alteration in granulocyte kinetics.