Nilsson I M
Thromb Haemost. 1976 Jun 30;35(3):510-21.
The incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophila B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40% have severe, 18% moderate, and 42% mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B. There are 2 main Haemophilia Centres (Stockholm, Malmö) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction I-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Suffcient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 millino units of factor IX are given per year. Treatment is free of charge. Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4-18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40-50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.
瑞典（总人口810万）现存活血友病患者的发病率约为男性1:15,000，全体人口1:30,000。1931年至1975年（6月）期间，瑞典每5年出生的血友病患者数量几乎保持不变。瑞典血友病家庭总数为284个（血友病A占77%，血友病B占23%），共有557名现存活血友病患者（血友病A 436名，血友病B 121名）。血友病A患者中，40%为重型，18%为中型，42%为轻型。血友病B患者的分布情况大致相同。重型血友病A患者中有8%、重型血友病B患者中有10%检测到有抑制物。瑞典有2个主要的血友病中心（斯德哥尔摩、马尔默），全瑞典的血友病患者都被收治于此，以诊断、随访并治疗严重出血、关节缺陷及手术。轻微出血则在当地医院与血友病中心合作进行治疗。可用于治疗血友病A的浓缩剂有人凝血因子I-0（AHF-卡比）、冷沉淀、抗血友病因子（海兰4）和冷球蛋白（免疫公司，维也纳）。AHF-卡比是最常用的制剂。可用于治疗血友病B的浓缩剂有普雷科纳蒂（卡比）和凝血酶原复合物（免疫公司）。浓缩剂供应充足。瑞典每年使用320万单位的凝血因子VIII和100万单位的凝血因子IX。治疗免费。只有5名患者接受家庭治疗，但自1958年以来，瑞典对4至18岁的重型血友病A男孩实施了预防性治疗。他们每隔约5至10天接受一次AHF，剂量足以将AHF水平提高到40%至50%。这种治疗方案已将重型血友病转变为中型血友病。关节评分与同年龄组中型血友病患者相同。对于合并有抑制物的血友病A和血友病B患者，我们采用大剂量抗原（凝血因子VIII或凝血因子IX）联合环磷酰胺进行治疗。在大多数情况下，这种治疗可产生5至30天的满意止血效果，并防止继发性抗体升高。
