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Bone-marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968-1985.
Lancet. 1986 Nov 08; 2(8515):1080-4.Lct

Abstract

In this retrospective analysis of allogeneic bone-marrow transplantation (BMT) carried out between 1969 and 1985 at fourteen European centres in 162 patients with sixteen different types of inherited immunodeficiencies and osteopetrosis, the overall survival with functional grafts was 51.7% (85 patients), with a minimum follow-up of 5 months. In patients with severe combined immunodeficiency HLA-matched (n = 41) and T-cell-depleted HLA-mismatched BMT (n = 46) resulted in 68% and 57% disease-free survival, respectively; after HLA-mismatched transplants, older age (greater than 6 months) and adenosine-deaminase deficiency resulted in poorer survival. Eight other lethal immunodeficiencies, including profound T-cell deficiencies, Wiskott-Aldrich syndrome, Kostmann syndrome, LFA-1/CR 3/p150,95 deficiency, and Chediak-Higashi syndrome as well as malignant osteopetrosis, have been successfully treated by BMT. In this group, survival with functional graft was 47% with HLA-matched and 29% with T-cell-depleted HLA-mismatched BMT. Engraftment failure was the major complication in this group. Poorer prognosis was associated with older patients, profound T-cell deficiencies, and the degree of HLA incompatibility.

Authors

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Pub Type(s)

Clinical Trial
Journal Article

Language

eng

PubMed ID

2877234

Citation

Fischer, A, et al. "Bone-marrow Transplantation for Immunodeficiencies and Osteopetrosis: European Survey, 1968-1985." Lancet (London, England), vol. 2, no. 8515, 1986, pp. 1080-4.
Fischer A, Griscelli C, Friedrich W, et al. Bone-marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968-1985. Lancet. 1986;2(8515):1080-4.
Fischer, A., Griscelli, C., Friedrich, W., Kubanek, B., Levinsky, R., Morgan, G., Vossen, J., Wagemaker, G., & Landais, P. (1986). Bone-marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968-1985. Lancet (London, England), 2(8515), 1080-4.
Fischer A, et al. Bone-marrow Transplantation for Immunodeficiencies and Osteopetrosis: European Survey, 1968-1985. Lancet. 1986 Nov 8;2(8515):1080-4. PubMed PMID: 2877234.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Bone-marrow transplantation for immunodeficiencies and osteopetrosis: European survey, 1968-1985. AU - Fischer,A, AU - Griscelli,C, AU - Friedrich,W, AU - Kubanek,B, AU - Levinsky,R, AU - Morgan,G, AU - Vossen,J, AU - Wagemaker,G, AU - Landais,P, PY - 1986/11/8/pubmed PY - 1986/11/8/medline PY - 1986/11/8/entrez SP - 1080 EP - 4 JF - Lancet (London, England) JO - Lancet VL - 2 IS - 8515 N2 - In this retrospective analysis of allogeneic bone-marrow transplantation (BMT) carried out between 1969 and 1985 at fourteen European centres in 162 patients with sixteen different types of inherited immunodeficiencies and osteopetrosis, the overall survival with functional grafts was 51.7% (85 patients), with a minimum follow-up of 5 months. In patients with severe combined immunodeficiency HLA-matched (n = 41) and T-cell-depleted HLA-mismatched BMT (n = 46) resulted in 68% and 57% disease-free survival, respectively; after HLA-mismatched transplants, older age (greater than 6 months) and adenosine-deaminase deficiency resulted in poorer survival. Eight other lethal immunodeficiencies, including profound T-cell deficiencies, Wiskott-Aldrich syndrome, Kostmann syndrome, LFA-1/CR 3/p150,95 deficiency, and Chediak-Higashi syndrome as well as malignant osteopetrosis, have been successfully treated by BMT. In this group, survival with functional graft was 47% with HLA-matched and 29% with T-cell-depleted HLA-mismatched BMT. Engraftment failure was the major complication in this group. Poorer prognosis was associated with older patients, profound T-cell deficiencies, and the degree of HLA incompatibility. SN - 0140-6736 UR - https://www.unboundmedicine.com/medline/citation/2877234/Bone_marrow_transplantation_for_immunodeficiencies_and_osteopetrosis:_European_survey_1968_1985_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0140-6736(86)90477-0 DB - PRIME DP - Unbound Medicine ER -