PMID: 7537525Mar 1, 1995Paper

Diamond-Blackfan anaemia: three patterns of in vitro response to haemopoietic growth factors

British Journal of Haematology
C P McGuckinE C Gordon-Smith

Abstract

Culture of bone marrow from patients with Diamond-Blackfan anaemia (DBA) has previously shown a variable progenitor response to growth factor stimulation. An extensive standardized study has now been undertaken to investigate the presence of distinct sub-groups in this disorder. In vitro response of bone marrow progenitors to recombinant human growth factors, including stem cell factor, was examined in 18 DBA patients and five normal donors, assessing BFU-E, CFU-GM and CFU-GEMM development. In 16 of the DBA patients a synergistic response to combinations of growth factors was observed with optimal growth in cultures containing erythropoietin, interleukin-3 and stem cell factor. Growth factor induced erythroid response formed three distinct groups, based on BFU-E numbers: type I (mean age 4.87 years) showed > 70% normal erythroid response; type II (mean age 13.87 years) showed < 70% normal; and type III (mean age 15.29 years) < 5% normal. CFU-GM response also followed the trigrouping. The results suggest more than one pathogenic mechanism for the erythroid failure in DBA, indicating DBA may be composed of more than one distinct disorder, and further suggest the defect in DBA may not be confined to the erythroid series.

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Citations

Jul 10, 2003·Blood Cells, Molecules & Diseases·Jan ZivnyTomas Stopka
Nov 25, 2000·British Journal of Haematology·C A SieffD G Nathan
Mar 28, 2001·Ryōikibetsu shōkōgun shirīzu·M Teramura
Mar 4, 2000·Current Opinion in Hematology·T N WilligC A Sieff
Oct 7, 2000·Paediatric Drugs·I DianzaniU Ramenghi
Nov 19, 2013·Pediatric Clinics of North America·S Deborah Chirnomas, Gary M Kupfer
Jul 27, 2010·Transfusion clinique et biologique : journal de la Société française de transfusion sanguine·L Da CostaT Leblanc
Sep 1, 2006·British Journal of Haematology·Hanna T Gazda, Colin A Sieff
Jan 27, 1998·Hematology/oncology Clinics of North America·O I Krijanovski, C A Sieff
Jan 1, 1998·Hematology·M H Mangi, A C Newland
Oct 1, 1995·Lupus·P D KielyJ C Marsh
Feb 13, 1999·Nature Genetics·N DraptchinskaiaN Dahl
Apr 17, 1998·Journal of Pediatric Hematology/oncology·E S GussetisS Graphakos
Mar 29, 2014·Expert Review of Hematology·Adrianna VlachosJeffrey M Lipton
Feb 13, 2001·British Journal of Haematology·P FischH E Schaefer
Nov 26, 2002·Hematology·Alan D D'AndreaAkiko Shimamura

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