Congenital Bleeding Disorders: Managing Central Nervous System Bleeding in an Adult Hemophiliac

先天性出血性疾病:成人血友病患者中枢神经系统出血的治疗

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Abstract

Congenital bleeding disorders remain a challenge to healthcare in the developing world. Despite the initiation of gene therapy almost five decades ago, the natural history of hemophilia remains the same. The cost of concentrated plasma factors, the development of a high titer of inhibitors in severe hemophilia A (HA), and the associated enhanced propensity of ICH make advancements in disease management questionable. Severe cases of hemophilia die young due to spontaneous central nervous system bleeds due to the lack of standard guidelines for plasma concentrate replacement and the limited availability of products due to the associated economic burden. Monoclonal antibodies, although a promising option as a standardized prophylactic treatment, remain underutilized due to availability and accessibility issues. Here, we report the case of a 28-year-old male with HA who presented to the emergency with a progressively worsening headache, nausea, and elevated blood pressure. He had an intracerebral hemorrhage (ICH) successfully managed with decongestants and factor VIII supplementation.

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