Klamroth R, Orlovic M, Kubicek-Hofman C, Gottstein S
Klinik für Innere Medizin - Angiologie und Hämostaseologie, Vivantes - Klinikum im Friedrichshain, Landsberger Allee 49, 10249 Berlin.
Hamostaseologie. 2010 Nov;30 Suppl 1:S26-7.
Haemophilia B (HB) was described in 1952 as a single disease for the first time. In comparison to haemophilia A (HA) the bleeding tendency seemed to be less severe. The aim of this study was to investigate this hypothesis in all patients with HA and HB treated in the haemophilia care center of the Vivantes Klinikum.
PATIENTS, METHODS: All patients with severe HA and HB treated at the haemophilia care center were included. We evaluated the regimen of replacement therapy and factor concentrate consumption within the last 5 years (1/2004 to 12/2008). Intracerebral bleeds were analysed over the whole life span of the included patients.
111/181 patients with HA had the severe form and 12/34 patients severe HB. 4/12 patients with severe HB had a history of intracerebral bleeding in comparison to 5/111 patients with severe HA. 2/8 adult patients with severe HB used a prophylactic treatment with factor concentrates (mean consumption 1289 IU factor IX/kg BW/year) in contrast to 60/95 adult patients with HA (mean consumption 2109 IU factor VIII /kg BW/year).
The data suggest a milder bleeding type of patients with severe HB in comparison to patients with severe HA but may be patients with severe HB are at higher risk for intracerebral bleeds.
1952年首次将血友病B（HB）描述为一种单一疾病。与血友病A（HA）相比，其出血倾向似乎没那么严重。本研究的目的是在维万特斯临床医院血友病护理中心治疗的所有HA和HB患者中验证这一假设。
患者、方法：纳入在血友病护理中心接受治疗的所有重度HA和HB患者。我们评估了过去5年（2004年1月至2008年12月）的替代治疗方案和凝血因子浓缩剂消耗量。对纳入患者的整个生命周期内的脑出血情况进行了分析。
181例HA患者中有111例为重度，34例HB患者中有12例为重度。12例重度HB患者中有4例有脑出血病史，而111例重度HA患者中有5例有脑出血病史。8例成年重度HB患者中有2例采用凝血因子浓缩剂预防性治疗（平均消耗量为1289 IU IX因子/kg体重/年），相比之下，95例成年HA患者中有60例采用预防性治疗（平均消耗量为2109 IU VIII因子/kg体重/年）。
数据表明，与重度HA患者相比，重度HB患者的出血类型较轻，但重度HB患者可能发生脑出血的风险更高。
