1999
DOI: 10.1016/s0301-472x(98)00068-x
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Long-term bone marrow cultures in Diamond-Blackfan anemia reveal a defect of both granulomacrophage and erythroid progenitors

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Cited by 35 publications
(19 citation statements)
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“…As a consequence, hemosiderosis is a major cause of death among transfusiondependent patients with DBA. In vitro hematopoietic progenitor culture studies indicate that DBA results from an intrinsic defect in erythroid progenitors, erythroid burst-forming units (BFU-Es) and erythroid colony-forming units (CFU-Es), 5,13 and not from a defect in the bone marrow microenvironment, 14 which is consistent with successful marrow transplantation as a treatment for DBA.…”
Section: Introductionmentioning
confidence: 83%
“…As a consequence, hemosiderosis is a major cause of death among transfusiondependent patients with DBA. In vitro hematopoietic progenitor culture studies indicate that DBA results from an intrinsic defect in erythroid progenitors, erythroid burst-forming units (BFU-Es) and erythroid colony-forming units (CFU-Es), 5,13 and not from a defect in the bone marrow microenvironment, 14 which is consistent with successful marrow transplantation as a treatment for DBA.…”
Section: Introductionmentioning
confidence: 83%
“…2,[16][17][18][19] A similar age-dependent decrease in granulocyte-macrophage progenitor (GMP) numbers has been reported. 20 Although present at normal frequencies, the proliferation and differentiation of immature hematopoietic progenitors in DBA have shown to be impaired in vitro. 2,18,20,21 Generation of animal models for RPS19-deficient DBA is pivotal to understand the disease mechanisms and to evaluate novel therapies.…”
Section: Introductionmentioning
confidence: 99%
“…(Chan, Saunders et al 1982) DBA CD34+ cells had impaired ability also to undergo granulocytic-monocytic differentiation in addition to their erythroid differentiation defect. (Santucci, Bagnara et al 1999) These results underscore a stem cell defect in DBA rather than an isolated erythroid defect. This is in keeping with the clinical observation that in addition to anemia patients can have neutropenia and thrombocytopenia.…”
Section: Dyskeratosis Congenita Genesmentioning
confidence: 67%