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凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物

OBJECTIVE: To explore the immune tolerance induction (ITI) in a case of severe hemophilia B patient with inhibitor. METHODS: The F Ⅸ∶C was detected using a one-stage method and FIX inhibitor was assayed using Bethesda method. ITI was performed with prothrombin complex concentrates (PCC) in combinati...

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Formato: Online Artículo Texto
Lenguaje:English
Publicado: Editorial office of Chinese Journal of Hematology 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7348358/
https://www.ncbi.nlm.nih.gov/pubmed/29081190
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.09.004
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description OBJECTIVE: To explore the immune tolerance induction (ITI) in a case of severe hemophilia B patient with inhibitor. METHODS: The F Ⅸ∶C was detected using a one-stage method and FIX inhibitor was assayed using Bethesda method. ITI was performed with prothrombin complex concentrates (PCC) in combination with rituximab. RESULTS: His past exposure days (ED) with PCC were 20 ED and his peak FⅨ inhibitor titer was 56 BU/ml. When his FIX inhibitor titer decreased to 10.4 BU/ml in Nov. 2015 and after receiving the informed consent from his parents, ITI was started. PCC with low dose rituximab successfully eradicated the high titer inhibitor within 17 months. There was no anaphylaxis, thrombotic event and infection. CONCLUSION: This is the first case report for successful immune tolerance induction therapy in Chinese hemophilia B patient. ITI using PCC combined with rituximab is an effective choice to induce immune tolerance of hemophilia B with inhibitor.
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spelling pubmed-73483582020-07-16 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物 Zhonghua Xue Ye Xue Za Zhi 论著 OBJECTIVE: To explore the immune tolerance induction (ITI) in a case of severe hemophilia B patient with inhibitor. METHODS: The F Ⅸ∶C was detected using a one-stage method and FIX inhibitor was assayed using Bethesda method. ITI was performed with prothrombin complex concentrates (PCC) in combination with rituximab. RESULTS: His past exposure days (ED) with PCC were 20 ED and his peak FⅨ inhibitor titer was 56 BU/ml. When his FIX inhibitor titer decreased to 10.4 BU/ml in Nov. 2015 and after receiving the informed consent from his parents, ITI was started. PCC with low dose rituximab successfully eradicated the high titer inhibitor within 17 months. There was no anaphylaxis, thrombotic event and infection. CONCLUSION: This is the first case report for successful immune tolerance induction therapy in Chinese hemophilia B patient. ITI using PCC combined with rituximab is an effective choice to induce immune tolerance of hemophilia B with inhibitor. Editorial office of Chinese Journal of Hematology 2017-09 /pmc/articles/PMC7348358/ /pubmed/29081190 http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.09.004 Text en 2017年版权归中华医学会所有 http://creativecommons.org/licenses/by-nc-sa/3.0/ This work is licensed under a Creative Commons Attribution 3.0 License (CC-BY-NC). The Copyright own by Publisher. Without authorization, shall not reprint, except this publication article, shall not use this publication format design. Unless otherwise stated, all articles published in this journal do not represent the views of the Chinese Medical Association or the editorial board of this journal.
spellingShingle 论著
凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物
title 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物
title_full 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物
title_fullStr 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物
title_full_unstemmed 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物
title_short 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病B伴抑制物
title_sort 凝血酶原复合物联合小剂量利妥昔单抗治疗血友病b伴抑制物
topic 论著
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7348358/
https://www.ncbi.nlm.nih.gov/pubmed/29081190
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2017.09.004
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