{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T05:24:38Z","timestamp":1777785878982,"version":"3.51.4"},"reference-count":10,"publisher":"FapUNIFESP (SciELO)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Einstein (S\u00e3o Paulo)"],"published-print":{"date-parts":[[2011,6]]},"abstract":"<jats:p> Objective: To report the experience of a tertiary care hospital with allogeneic hematopoietic stem cell transplantation in children with primary immunodeficiencies.  Methods: Seven pediatric patients with primary immunodeficiencies (severe combined immunodeficiency: n = 2; combined immunodeficiency: n = 1; chronic granulomatous disease: n = 1; hyper-IgM syndrome: n = 2; and IPEX syndrome: n = 1) who underwent eight hematopoietic stem cell transplants in a single center, from 2007 to 2010, were studied.  Results: Two patients received transplants from HLA-identical siblings; the other six transplants were done with unrelated donors (bone marrow: n = 1; cord blood: n = 5). All patients had pre-existing infections before hematopoietic stem cell transplants. One patient received only anti-thymocyte globulin prior to transplant, three transplants were done with reduced intensity conditioning regimens and four transplants were done after myeloablative therapy. Two patients were not evaluated for engraftment due to early death. Three patients engrafted, two had primary graft failure and one received a second transplant with posterior engraftment. Two patients died of regimen related toxicity (hepatic sinusoidal obstruction syndrome); one patient died of progressive respiratory failure due to Parainfluenza infection present prior to transplant. Four patients are alive and well from 60 days to 14 months after transplant.  Conclusion: Patients\u2019 status prior to transplant is the most important risk factor on the outcome of hematopoietic stem cell transplants in the treatment of these diseases. Early diagnosis and the possibility of a faster referral of these patients for treatment in reference centers may substantially improve their survival and quality of life.<\/jats:p>","DOI":"10.1590\/s1679-45082011ao2007","type":"journal-article","created":{"date-parts":[[2015,9,23]],"date-time":"2015-09-23T22:53:20Z","timestamp":1443048800000},"page":"140-144","source":"Crossref","is-referenced-by-count":4,"title":["Allogeneic hematopoietic stem cell transplantation in children with primary immunodeficiencies: Hospital Israelita Albert Einstein experience"],"prefix":"10.1590","volume":"9","author":[{"given":"Juliana Folloni","family":"Fernandes","sequence":"first","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabio Rodrigues","family":"Kerbauy","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreza Alice Feitosa","family":"Ribeiro","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jose Mauro","family":"Kutner","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luis Fernando Aranha","family":"Camargo","sequence":"additional","affiliation":[{"name":"Instituto Israelita de Ensino e Pesquisa Albert Eisntein - IIEPAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adalberto","family":"Stape","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eduardo Juan","family":"Troster","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriele","family":"Zamperlini-Netto","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra Milani Prandini de","family":"Azambuja","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruna","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mayra de Barros","family":"Dorna","sequence":"additional","affiliation":[{"name":"Unversidade de S\u00e3o Paulo - USP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marluce dos Santos","family":"Vilela","sequence":"additional","affiliation":[{"name":"Universidade Estadual de Campinas - UNICAMP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristina Miuki Abe","family":"Jacob","sequence":"additional","affiliation":[{"name":"Unversidade de S\u00e3o Paulo - USP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatriz Tavares","family":"Costa-Carvalho","sequence":"additional","affiliation":[{"name":"Universidade Federal de S\u00e3o Paulo - UNIFESP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jose Marcos","family":"Cunha","sequence":"additional","affiliation":[{"name":"Universidade Federal do Rio de Janeiro - UFRJ,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magda Maria","family":"Carneiro-Sampaio","sequence":"additional","affiliation":[{"name":"Unversidade de S\u00e3o Paulo - USP,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nelson","family":"Hamerschlak","sequence":"additional","affiliation":[{"name":"Hospital Israelita Albert Einstein - HIAE,  Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"530","reference":[{"issue":"4","key":"ref1","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1016\/j.jaci.2007.08.053","article-title":"International Union of Immunological Societies Primary Immunodeficiency Diseases Classification Committee. 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