{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:28:37Z","timestamp":1760189317102},"reference-count":22,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,5,29]],"date-time":"2013-05-29T00:00:00Z","timestamp":1369785600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2013,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Partial albinism with variable immunodeficiency are the two major characteristics of<jats:styled-content style=\"fixed-case\">G<\/jats:styled-content>riscelli syndrome type 2 (<jats:styled-content style=\"fixed-case\">GS<\/jats:styled-content>\u20102). This syndrome is usually associated with a high mortality rate and commonly results in early childhood death. Patients suffer from different infections and experience crisis of<jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>.<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>remains the sole curative treatment for<jats:styled-content style=\"fixed-case\">GS<\/jats:styled-content>\u20102. We prospectively analyzed the outcomes of transplantation with<jats:styled-content style=\"fixed-case\">RIC<\/jats:styled-content>regimen in five patients. The median age at transplantation was 21.6\u00a0months (range: 12\u201330). All of the patients underwent<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>from<jats:styled-content style=\"fixed-case\">HLA<\/jats:styled-content>\u2010matched related donors. Currently, four patients are cured, and symptoms of recurrent infections and<jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>crisis are not seen in them. The only patient who died had undergone<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>in the accelerated phase of<jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>. One patient who developed acute<jats:styled-content style=\"fixed-case\">GvHD<\/jats:styled-content>had a favorable response to therapy. No chronic<jats:styled-content style=\"fixed-case\">GvHD<\/jats:styled-content>occurred in patients. It seems that the use of<jats:styled-content style=\"fixed-case\">RIC<\/jats:styled-content>regimen as a method of transplant preparation is effective and tolerable in this group of patients with various comorbidities. It is recommended to carry out<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>in these patients at lower ages, before presentations of different infections and<jats:styled-content style=\"fixed-case\">HLH<\/jats:styled-content>crisis.<\/jats:p>","DOI":"10.1111\/petr.12092","type":"journal-article","created":{"date-parts":[[2013,5,29]],"date-time":"2013-05-29T07:14:01Z","timestamp":1369811641000},"page":"487-491","source":"Crossref","is-referenced-by-count":13,"title":["Hematopoietic stem cell transplantation with a reduced\u2010intensity conditioning regimen in pediatric patients with<scp>G<\/scp>riscelli syndrome type 2"],"prefix":"10.1111","volume":"17","author":[{"given":"Amir Ali","family":"Hamidieh","sequence":"first","affiliation":[{"name":"Hematology\u2010Oncology and Stem Cell Transplantation Research Center, Tehran University of Medical Sciences Tehran 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