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.
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IX因子缺乏引起。获得性A型血友病是一种罕见的,获得性出血性疾病表现为新发出血,尤其是老年人,由于抗因子VIII(FVIII)的自身抗体的发展。本病例报告介绍了先天性血友病B和获得性血友病A患者的医疗管理。我们强调了单独使用因子替代疗法维持因子水平的局限性。特别是在开发因子抑制剂的血友病患者中。此外,我们提请注意剂量增加的必要性,成本,以及免疫耐受诱导治疗的需要。此病例说明,当当前的诊断不能解释完整的临床表现和实验室数据不充分时,继续寻求替代诊断和具有成本效益的治疗非常重要.