Beddall A C, Al-Rubei K, Williams M D, Hill F G
Arch Dis Child. 1985 Jun;60(6):530-6. doi: 10.1136/adc.60.6.530.
Type and quantity of replacement treatment, together with haematological and immunological parameters were determined in 37 boys with severe haemophilia A and 41 children with other bleeding disorders. The quantity of factor VIII concentrate given to boys with severe haemophilia A (mean U/year) showed a significant inverse correlation with total white cell counts, lymphocyte counts, platelet counts, and the ratio of monoclonal antibody defined T lymphocyte subsets, T4 and T8 (T4:T8). Of the boys with severe haemophilia A, 49% had inversed T4:T8 ratios and 24% had thrombocytopenia. Treatment with high dose factor VIII concentrate (more than 25 000 U/year) was associated with low platelet counts, low lymphocyte counts, low T4:T8 ratios, and hypergammaglobulinaemia. In addition, six patients with severe haemophilia A and factor VIII inhibitors had inversed T4:T8 ratios. Patients treated exclusively with cryoprecipitate or prothrombin complex concentrates had normal T4:T8 ratios and platelet counts. The severity of the haematological and immunological abnormalities observed seems to be associated with high usage of factor VIII concentrates. Similar abnormalities have been described in patients with the acquired immune deficiency syndrome (AIDS). Prospective study of haemophiliacs is required to assess long term sequelae of factor concentrate usage, including the possible development of AIDS.
对37名重度甲型血友病男孩和41名患有其他出血性疾病的儿童测定了替代治疗的类型和剂量，以及血液学和免疫学参数。给予重度甲型血友病男孩的凝血因子VIII浓缩物剂量（平均单位/年）与白细胞总数、淋巴细胞计数、血小板计数以及单克隆抗体定义的T淋巴细胞亚群T4和T8的比例（T4:T8）呈显著负相关。在重度甲型血友病男孩中，49%的患者T4:T8比例倒置，24%的患者有血小板减少症。高剂量凝血因子VIII浓缩物治疗（每年超过25000单位）与血小板计数低、淋巴细胞计数低、T4:T8比例低和高丙种球蛋白血症有关。此外，6名患有重度甲型血友病且有凝血因子VIII抑制物的患者T4:T8比例倒置。仅接受冷沉淀或凝血酶原复合物浓缩物治疗的患者T4:T8比例和血小板计数正常。观察到的血液学和免疫学异常的严重程度似乎与凝血因子VIII浓缩物的高使用量有关。在获得性免疫缺陷综合征（AIDS）患者中也描述了类似的异常情况。需要对血友病患者进行前瞻性研究，以评估凝血因子浓缩物使用的长期后遗症，包括可能发生的艾滋病。
