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。

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
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引用次数: 0

摘要

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

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.

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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
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