Hemopoietic marrow function in chronic neutropenia of blacks: Cure of aplastic anemia by allogeneic marrow transplantation from a neutropenic sibling donor

Abstract

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 family 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.

Analysis of overall survival in a large multiethnic cohort reveals absolute neutrophil count of 1,100 as a novel prognostic cutoff in African Americans

“…Although a benign physiology and the absence of susceptibility to infections, has been well documented in the literature [8-10, 13, 14], the reference range for neutrophils used in clinical practice is the same across races and ethnicities. As such, individuals primarily of African ancestry are diagnosed with neutropenia that often represents the so-called benign ethnic neutropenia, yet frequently undergo extensive hematologic work-up, especially in the elderly.…”

Genetics, phenotype, and natural history of autosomal dominant cyclic hematopoiesis

“…ANCs range between 1,000 and 2,000/mm3. It is more common in Middle Eastern, Yemenite Jewish, and African ethnic groups [Ash et al, 1986;Shoenfeld et al, 19881. 3) Congenital neutropenias: Other families with neutropenia clearly do not fit the above two groups. In addition to ADCH, we propose the following categories, which may be genetically distinct groups: [Falk et al, 1976;Joyce et al, 1977;Kirstila et al, 19931.…”