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Titolo:
Hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency
Autore:
Buckley, RH; Schiff, SE; Schiff, RI; Markert, ML; Williams, LW; Roberts, JL; Myers, LA; Ward, FE;
Indirizzi:
Duke Univ, Med Ctr, Dept Pediat, Durham, NC 27710 USA Duke Univ Durham NCUSA 27710 Med Ctr, Dept Pediat, Durham, NC 27710 USA Duke Univ, Med Ctr, Dept Immunol, Durham, NC 27710 USA Duke Univ Durham NC USA 27710 Med Ctr, Dept Immunol, Durham, NC 27710 USA
Titolo Testata:
NEW ENGLAND JOURNAL OF MEDICINE
fascicolo: 7, volume: 340, anno: 1999,
pagine: 508 - 516
SICI:
0028-4793(19990218)340:7<508:HSTFTT>2.0.ZU;2-U
Fonte:
ISI
Lingua:
ENG
Soggetto:
BONE-MARROW TRANSPLANTATION; IN-UTERO TRANSPLANTATION; SOYBEAN LECTIN; BLOOD; RECONSTITUTION; DISORDERS; DISEASES; CHILDREN; MUTATION; INFANTS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
44
Recensione:
Indirizzi per estratti:
Indirizzo: Buckley, RH Duke Univ, Med Ctr, Dept Pediat, Box 2898, Durham, NC 27710 USA Duke Univ Box 2898 Durham NC USA 27710 8, Durham, NC 27710 USA
Citazione:
R.H. Buckley et al., "Hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency", N ENG J MED, 340(7), 1999, pp. 508-516

Abstract

Background Since 1968 it has been known that bone marrow transplantation can ameliorate severe combined immunodeficiency, bur data on the longterm efficacy of this treatment are limited. We prospectively studied immunologic function in 89 consecutive infants with severe combined immunodeficiency who received hematopoietic stem-cell transplants at Duke University Medical Center between May 1982 and September 1998. Methods Serum immunoglobulin levels and lymphocyte phenotypes and functionwere assessed and genetic analyses performed according to standard methods. Bone marrow was depleted of T cells by agglutination with soybean lectin and by sheep-erythrocyte resetting before transplantation. Results Seventy-seven of the infants received T-cell-depleted, HLA-haploidentical parental marrow, and 12 received HLA-identical marrow from a related donor; 3 of the recipients of haploidentical marrow also received placental-blood transplants from unrelated donors. Except for two patients who received placental blood, none of the recipients received chemotherapy before transplantation or prophylaxis against graft-versus-host: disease. Of the 89 infants, 72 (81 percent) were still alive 3 months to 16.5 years after transplantation, including all of the 12 who received HLA-identical marrow, 60 of the 77 (78 percent) who were given haploidentical marrow, and 2 of the3 (67 percent) who received both haploidentical marrow and placental blood. T-cell function became normal within two weeks after transplantation in the patients who received unfractionated HLA-identical marrow but usually not until three to four months after transplantation in those who received T-cell-depleted marrow. At the time of the most recent evaluation, all but 4 of the 72 survivors had normal T-cell function, and all the T cells in their blood were of donor origin. B-cell function remained abnormal in many of the recipients of haploidentical marrow. In 26 children (5 recipients of HLA-identical marrow and 21 recipients of haploidentical marrow) between 2 percent and 100 percent of B cells were of donor origin. Forty-five of the 72children were receiving intravenous immune globulin. Conclusions Transplantation of marrow from a related donor is a life-saving and life-sustaining treatment for patients with any type of severe combined immunodeficiency, even when there is no HLA-identical donor. (N Engl J Med 1999;340:508-16. ) (C)1999. Massachusetts Medical Society.

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Documento generato il 27/11/20 alle ore 22:14:00