Mishra P, Naithani R, Dolai T, Bhargava R, Mahapatra M, Dixit A, Seth T, Kumar R, Saxena R
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.
Haemophilia. 2008 Sep;14(5):952-5. doi: 10.1111/j.1365-2516.2008.01814.x. Epub 2008 Jul 14.
We investigated 52 of 457 patients with congenital factor deficiencies with 57 episodes of intracranial haemorrhage (ICH) between 1998 and 2007. There were 38 severe haemophiliacs, 6 with factor XIII deficiency, 5 with factor X deficiency, 2 factor V-deficient patients, and 1 with type 3 von Willebrand disease (VWD). The median age was 8 years (range 1 month-22 years). Most patients were below 15 years of age (86.5%). All patients with factor X deficiency were between 1 and 5 months of age. ICH was the primary bleeding episode leading to detection of factor deficiency in 19.2% (five patients with severe haemophilia and all patients with factor X deficiency). Trauma caused bleeding in 66%. None of the patients with factor X deficiency had history of prior trauma. Surgery was performed in five patients with subdural haematomas, all of whom survived. Conservative factor replacement with 100% correction for 3 days followed by 50-60% correction for 7 days was possible in 60% patients. Seizures requiring prolonged therapy were noted in eight patients. Death was recorded in 15 patients (29%). Inadequate therapy in the form of delay or insufficient replacement was noted in 7/15 deaths. ICH was seen in 11.3% of all patients with coagulation factor deficiencies. Factor X deficiency presented with ICH at an earlier age. Inadequate replacement therapy including delayed treatment caused nearly 50% of all deaths. Most patients can be managed satisfactorily with adequate replacement therapy alone, with surgery being reserved for those with worsening neurological conditions.
我们对457例先天性凝血因子缺乏症患者中的52例进行了调查，这些患者在1998年至2007年间发生了57次颅内出血（ICH）。其中有38例重度血友病患者，6例因子ⅩⅢ缺乏症患者，5例因子Ⅹ缺乏症患者，2例因子Ⅴ缺乏症患者，以及1例3型血管性血友病（VWD）患者。中位年龄为8岁（范围1个月至22岁）。大多数患者年龄在15岁以下（86.5%）。所有因子Ⅹ缺乏症患者年龄在1至5个月之间。ICH是导致19.2%的患者（5例重度血友病患者和所有因子Ⅹ缺乏症患者）被检测出凝血因子缺乏的主要出血事件。66%的出血是由创伤引起的。因子Ⅹ缺乏症患者均无既往创伤史。5例患有硬膜下血肿的患者接受了手术，所有患者均存活。60%的患者可行保守性凝血因子替代治疗，先100%纠正3天，然后50 - 60%纠正7天。8例患者出现需要长期治疗的癫痫发作。15例患者（29%）死亡。15例死亡患者中有7例存在治疗延迟或替代不足的情况。在所有凝血因子缺乏症患者中，ICH的发生率为11.3%。因子Ⅹ缺乏症患者发生ICH的年龄更早。包括治疗延迟在内的替代治疗不足导致了近50%的死亡。大多数患者仅通过充分的替代治疗即可得到满意的治疗效果，手术仅适用于神经状况恶化的患者。
