Concurrent congenital hemophilia B and acquired hemophilia A: a unique case report.

IF 1.2 4区 医学 Q4 HEMATOLOGY Blood Coagulation & Fibrinolysis Pub Date : 2024-07-01 Epub Date: 2024-05-01 DOI:10.1097/MBC.0000000000001306
Salih Cirik, Mehmet Ali Erkurt, İrfan Kuku, Emin Kaya, İlhami Berber, Emine Hidayet, Soykan Biçim, Ahmet Kaya, Süleyman Arslan, Ayşe Günay
{"title":"Concurrent congenital hemophilia B and acquired hemophilia A: a unique case report.","authors":"Salih Cirik, Mehmet Ali Erkurt, İrfan Kuku, Emin Kaya, İlhami Berber, Emine Hidayet, Soykan Biçim, Ahmet Kaya, Süleyman Arslan, Ayşe Günay","doi":"10.1097/MBC.0000000000001306","DOIUrl":null,"url":null,"abstract":"<p><p>Congenital hemophilia B is a rare X-linked recessive bleeding disorder caused by factor IX deficiency. Acquired hemophilia A is a rare, acquired bleeding disorder that presents with new-onset bleeding, especially in older adults, due to the development of auto-antibodies against factor VIII (FVIII). This case report presents the medical management of a patient with congenital hemophilia B and acquired hemophilia A. We highlight the limitations of maintaining factor levels with factor replacement therapy alone, particularly in hemophilia patients who have developed factor inhibitors. In addition, we draw attention to the need for dose escalation, the cost, and the need for immune-tolerance induction therapy. This case illustrates that when the current diagnosis does not explain the full clinical picture and laboratory data are inadequate, it is important to continue to seek alternative diagnoses and cost-effective treatment.</p>","PeriodicalId":8992,"journal":{"name":"Blood Coagulation & Fibrinolysis","volume":null,"pages":null},"PeriodicalIF":1.2000,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Blood Coagulation & Fibrinolysis","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1097/MBC.0000000000001306","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/5/1 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Congenital hemophilia B is a rare X-linked recessive bleeding disorder caused by factor IX deficiency. Acquired hemophilia A is a rare, acquired bleeding disorder that presents with new-onset bleeding, especially in older adults, due to the development of auto-antibodies against factor VIII (FVIII). This case report presents the medical management of a patient with congenital hemophilia B and acquired hemophilia A. We highlight the limitations of maintaining factor levels with factor replacement therapy alone, particularly in hemophilia patients who have developed factor inhibitors. In addition, we draw attention to the need for dose escalation, the cost, and the need for immune-tolerance induction therapy. This case illustrates that when the current diagnosis does not explain the full clinical picture and laboratory data are inadequate, it is important to continue to seek alternative diagnoses and cost-effective treatment.

查看原文
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
并发先天性血友病 B 和后天性血友病 A:一份独特的病例报告。
先天性血友病 B 是一种罕见的 X 连锁隐性出血性疾病,由因子 IX 缺乏引起。后天性血友病 A 是一种罕见的后天性出血性疾病,由于产生了针对因子 VIII (FVIII) 的自身抗体,患者会出现新发出血,尤其是老年人。本病例报告介绍了对一名先天性血友病 B 和后天性血友病 A 患者的医学治疗。我们强调了仅靠因子替代疗法维持因子水平的局限性,尤其是对已出现因子抑制剂的血友病患者而言。此外,我们还提请注意剂量升级的必要性、成本以及免疫耐受诱导疗法的必要性。这个病例说明,当目前的诊断无法解释全部临床情况,实验室数据也不充分时,必须继续寻求其他诊断和具有成本效益的治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 去求助
来源期刊
CiteScore
1.90
自引率
0.00%
发文量
111
审稿时长
4-8 weeks
期刊介绍: Blood Coagulation & Fibrinolysis is an international fully refereed journal that features review and original research articles on all clinical, laboratory and experimental aspects of haemostasis and thrombosis. The journal is devoted to publishing significant developments worldwide in the field of blood coagulation, fibrinolysis, thrombosis, platelets and the kininogen-kinin system, as well as dealing with those aspects of blood rheology relevant to haemostasis and the effects of drugs on haemostatic components
期刊最新文献
Is the combination of two automated rapid assays for diagnosis of heparin-induced thrombocytopenia necessary? Variation among venous thromboembolism risk assessment tools for postcesarean patients: a retrospective cohort study. The role of factor XIII in patient blood management. Severe acquired Factor VII deficiency complicating an aplastic anemia, successfully treated with corticosteroids. Improved prevention of bleeding episodes with emicizumab in 3 patients with concomitant hemophilia A and von Willebrand disease.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1