{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T16:28:03Z","timestamp":1787761683098,"version":"build-2784847793"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,12,20]],"date-time":"2024-12-20T00:00:00Z","timestamp":1734652800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2024,12,20]],"date-time":"2024-12-20T00:00:00Z","timestamp":1734652800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp;amp; Cancer"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Primary hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by immune dysregulation. Hematopoietic stem cell transplantation (HSCT) represents the only option for long\u2010term cure for primary HLH. However, only around 25% of patients have a fully HLA\u2010matched donor.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      In this retrospective study, we analyzed 42 pediatric patients with primary HLH who underwent haplo\u2010SCT using the modified granulocyte colony\u2010stimulating factor (G\u2010CSF)\/antithymocyte globulin (ATG)\u2010based protocol. The conditioning regimen included 300\u2013600\u00a0mg\/m\n                      <jats:sup>2<\/jats:sup>\n                      etoposide (VP16), along with low doses of busulfan (Bu) (0.8\u20131.2\u00a0mg\/kg every 6\u00a0hours on Days \u22128 to \u22126), cyclophosphamide (Cy) (10\u00a0mg\/kg\/day on Days \u22124 to \u22123), fludarabine (Flu) (30\u00a0mg\/m\n                      <jats:sup>2<\/jats:sup>\n                      \/day on Days \u22125 to \u22123), and ATG (8\u20139\u00a0mg\/kg total dose on Days \u22125 to \u22122) to reduce complications.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      All 42 patients achieved successful engraftment. Following a median follow\u2010up period of 48.7\u00a0months, 32 of the 42 patients remained alive and disease free. The 2\u2010year overall survival (OS) rate was 78.4%, and the 5\u2010year OS rate was 73.7%. The 2\u2010year failure\u2010free survival (FFS) rate was 71.3%, and the 5\u2010year FFS rate was 66.5%. Patients who achieved complete remission at the time of HSCT showed better OS (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u00a00.05). The incidence of Grade III\u2013IV acute graft\u2010versus\u2010host disease (GVHD) was 26.2%, and severe chronic GVHD was observed in 11.9% of patients. Thrombotic microangiopathy occurred in 13 patients, and veno\u2010occlusive disease in two patients.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This modified G\u2010CSF\/ATG\u2010based haploidentical protocol demonstrates significant potential for pediatric patients with primary HLH, exhibiting commendable effectiveness and safety.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/pbc.31495","type":"journal-article","created":{"date-parts":[[2024,12,20]],"date-time":"2024-12-20T08:58:55Z","timestamp":1734685135000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Modified G\u2010CSF\/ATG\u2010Based Haploidentical Transplantation Protocol in Pediatric Primary Hemophagocytic Lymphohistiocytosis: A Long\u2010Term Follow\u2010Up Single\u2010Center Experience"],"prefix":"10.1002","volume":"72","author":[{"given":"Juan","family":"Xiao","sequence":"first","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9147-4791","authenticated-orcid":false,"given":"Xingcheng","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Hematology Tongji Hospital Tongji Medical College Huazhong University of Science and Technology  Wuhan Hubei China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nanhai","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shifen","family":"Fan","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhouyang","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8311-6302","authenticated-orcid":false,"given":"Fan","family":"Jiang","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiao","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jia","family":"Wei","sequence":"additional","affiliation":[{"name":"Department of Hematology Tongji Hospital Tongji Medical College Huazhong University of Science and Technology  Wuhan Hubei China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuan","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Hematology and Oncology Beijing Jingdu Children's Hospital  Beijing 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