Stem cell transplantation for primary immunodeficiencies: King Faisal Specialist Hospital experience from 1993 to 2006

Bone Marrow Transplantation
A Al-Ghonaium

Abstract

Primary immunodeficiencies constitute a group of highly complex congenital disorders most of which are characterized by poor prognosis with high mortality and morbidity. Hematopoietic SCT became an established therapy for such disorders. The first clear-cut report of successful allogenic SCT in 1968 dealt with the treatment of a patient with primary immunodeficiency, that is, SCID and Wiskott-Aldrich syndrome. Starting with this pioneering experience in 1968, hundreds of SCID patients and hundreds of patients affected by other life-threatening forms of primary immunodeficiency throughout the world have benefited from SCT. Presently, hematopoietic SCT from an HLA-matched sibling donor confers at least 80% chance of cure for children affected by primary immunodeficiency and about a 70% chance of cure when a fully HLA-matched related donor is available. This high success rate is the consequence of better management of nutrition and the infection problem affecting these patients at the time of disease. Conversely, when a related HLA-mismatched donor is used, the survival rate is significantly lower than that of patients receiving SCT from either an HLA-matched sibling or a fully matched HLA-unrelated donor. Optimal results and outc...Continue Reading

References

Jan 22, 2005·Annual Review of Medicine·Marina Cavazzana-CalvoAlain Fischer
Jun 6, 2007·The Journal of Clinical Investigation·Marina Cavazzana-Calvo, Alain Fischer

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Citations

Sep 25, 2012·Bone Marrow Transplantation·T N SmallM Sparber-Sauer
Dec 3, 2014·Clinical Immunology : the Official Journal of the Clinical Immunology Society·B M Jonkman-BerkT W Kuijpers
Jan 18, 2011·Hematology/oncology Clinics of North America·Eyal Grunebaum, Chaim M Roifman
May 25, 2010·Immunology and Allergy Clinics of North America·Sung-Yun Pai, Luigi D Notarangelo
Feb 2, 2010·Immunology and Allergy Clinics of North America·Eyal Grunebaum, Chaim M Roifman
Jan 2, 2010·Medicina clínica·Francisco José OrtuñoAndrés Jerez
Feb 4, 2012·Annals of the New York Academy of Sciences·Saleh Al-Muhsen, Zobaida Alsum
Jan 17, 2013·The Journal of Allergy and Clinical Immunology·Waleed Al-Herz, Hamoud Al-Mousa
Oct 20, 2009·Early Human Development·Arnalda LanfranchiGaetano Chirico

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