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231226s2022 xx |||||o 00| ||eng c |
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|a 10.1016/j.clim.2022.108993
|2 doi
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|a pubmed24n1129.xml
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|a (DE-627)NLM338972838
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|a (NLM)35367395
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|a (PII)S1521-6616(22)00074-2
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|a DE-627
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|e rakwb
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|a eng
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|a Tomomasa, Dan
|e verfasserin
|4 aut
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|a Preemptive hematopoietic cell transplantation for asymptomatic patients with X-linked lymphoproliferative syndrome type 1
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|c 2022
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|a Text
|b txt
|2 rdacontent
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|a ƒaComputermedien
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|2 rdamedia
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|a ƒa Online-Ressource
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|a Date Completed 18.04.2022
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|a Date Revised 20.05.2022
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|a published: Print-Electronic
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|a Citation Status MEDLINE
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|a Copyright © 2022. Published by Elsevier Inc.
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|a Few reports have examined whether prophylactic allogeneic hematopoietic cell transplantation (HCT) for X-linked lymphoproliferative syndrome type 1 (XLP1) improves the prognosis. We compared the prognosis of symptomatic probands and affected siblings in the same family. Twenty-two cases (10 probands and 12 affected siblings) in Japan, the United Kingdom, and the United States were analyzed. The overall survival (OS) rate at 5 years after diagnosis was 70.0% in probands and 91.7% in affected siblings (p = 0.0789). The prognosis of patients who developed symptoms of XLP1 before HCT and those who did not was also compared. The 5-year probability of OS from the time of diagnosis in asymptomatic patients (100%) was significantly better than that in symptomatic patients (66.7%). These results suggested that early HCT as soon as the diagnosis is made improves the prognosis in asymptomatic XLP1 patients
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|a Journal Article
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|a Preemptive hematopoietic cell transplantation
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|a X-linked lymphoproliferative syndrome type 1
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1 |
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|a Booth, Claire
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Bleesing, Jack J
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Isoda, Takeshi
|e verfasserin
|4 aut
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| 700 |
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|a Kobayashi, Chie
|e verfasserin
|4 aut
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| 700 |
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|a Koike, Kazutoshi
|e verfasserin
|4 aut
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| 700 |
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|a Taketani, Takeshi
|e verfasserin
|4 aut
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| 700 |
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|a Sawada, Akihisa
|e verfasserin
|4 aut
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| 700 |
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|a Tamura, Akihiro
|e verfasserin
|4 aut
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| 700 |
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|a Marsh, Rebecca A
|e verfasserin
|4 aut
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| 700 |
1 |
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|a Morio, Tomohiro
|e verfasserin
|4 aut
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| 700 |
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|a Gennery, Andrew R
|e verfasserin
|4 aut
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| 700 |
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|a Kanegane, Hirokazu
|e verfasserin
|4 aut
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| 773 |
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|i Enthalten in
|t Clinical immunology (Orlando, Fla.)
|d 1999
|g 237(2022) vom: 01. Apr., Seite 108993
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|g volume:237
|g year:2022
|g day:01
|g month:04
|g pages:108993
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