PMID: 7545424Aug 1, 1995Paper

Transplanted sickle-cell disease patients with autologous bone marrow recovery after graft failure develop increased levels of fetal haemoglobin which corrects disease severity

British Journal of Haematology
A FersterE Sariban

Abstract

Bone marrow transplantation (BMT) is the only curative therapy for sickle-cell disease (SCD), but is not devoid of failure risk. Nine patients with severe SCD were grafted in our institution between 1988 and 1993. Six patients successfully engrafted, but three failed to engraft and had delayed autologous recovery. All patients had, prior to BMT, low levels of fetal haemoglobin (HbF < or = 3.5%). No change in HbF occurred in successfully grafted patients. In the three patients with graft failure HbF increased and remained persistently present at a high level (> or = 22%) 14 months, 16 months and 39 months post BMT, although two of the three patients were homozygous for either the Benin or the Central African Republic haplotype, a characteristic associated with low HbF level. Of interest, these three previously severely affected patients remain free of vaso-occlusive events. The mechanism responsible for the expression of high levels of HbF in our three patients with graft failure is not understood, but it protects them from the recurrence of severe vaso-occlusive crises.

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Citations

Mar 19, 2003·Blood Reviews·Sally C Davies, Annette Gilmore
Oct 16, 1999·British Journal of Haematology·O Castro
May 3, 2000·British Journal of Haematology·D R Powars
May 4, 2012·Cold Spring Harbor Perspectives in Medicine·Guido LucarelliJavid Gaziev
Jul 1, 1996·Archives of Disease in Childhood·S C Davies, I A Roberts
Sep 2, 2014·Chemical & Pharmaceutical Bulletin·Feng RenNa Jin
Jun 15, 2011·Expert Review of Hematology·Ruby Khoury, Miguel R Abboud
Jan 26, 2007·Expert Opinion on Biological Therapy·Lakshmanan Krishnamurti
Nov 16, 2010·Hematology/oncology Clinics of North America·Vijay G Sankaran, David G Nathan
Mar 24, 2010·Pediatric Clinics of North America·Frans J SmiersGuido Lucarelli
Mar 5, 2003·British Journal of Haematology·Persis J AmroliaSally C Davies
Mar 5, 2003·British Journal of Haematology·Persis J AmroliaIrene A G Roberts
Oct 25, 2008·Journal of Hematology & Oncology·Che K LimWilliam Y K Hwang
Mar 31, 2016·Experimental Biology and Medicine·Winfred C Wang
Nov 19, 2019·The Journal of Clinical Investigation·Robert A Brodsky, Michael R DeBaun
Sep 9, 2020·The Journal of Clinical Investigation·Selami DemirciJohn F Tisdale

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