Alkjaersig N, Fletcher A, Joist H, Chaplin H
J Lab Clin Med. 1976 Sep;88(3):440-9.
Three patients with sickle-cell disease (SSD) were followed, weekly, for 1 1/2 to 2 years, during which time they experienced one or more episodes of crisis. Crisis was associated with reproducible sequential hemostatic alterations indicating intravascular fibrin formation and a marked disturbance in platelet economy. With crisis onset, or possibly before, there was an increase in plasma high-molecular-weight fibrinogen complexes and a transient fall in platelet count, with a subsequent rise in both fibrinogen concentration and platelet count; plasma fibrinogen peaked 1 week after crisis onset and platelet count approximately 2 weeks after onset. Subsidence of crisis was associated with a fall in high-molecular-weight fibrinogen complexes and a subsequent increase in fibrinogen first derivative, an early fibrinogen breakdown product. Hemostatic findings and patient clinical status were generally correlated, the findings during asymptomatic periods being essentially normal. Agents affecting platelet function (aspirin alone or in combination with dipyridamole) appeared to reduce the extent of laboratory abnormality, suggesting potential clinical usefulness in this disorder.
对三名镰状细胞病（SSD）患者进行了为期1年半至2年的每周随访，在此期间他们经历了一次或多次危机发作。危机与可重复的顺序性止血改变相关，表明血管内纤维蛋白形成以及血小板状态明显紊乱。在危机发作时或可能在发作之前，血浆高分子量纤维蛋白原复合物增加，血小板计数短暂下降，随后纤维蛋白原浓度和血小板计数均升高；血浆纤维蛋白原在危机发作后1周达到峰值，血小板计数在发作后约2周达到峰值。危机消退与高分子量纤维蛋白原复合物减少以及随后纤维蛋白原第一衍生物（一种早期纤维蛋白原降解产物）增加有关。止血结果与患者临床状态通常相关，无症状期的结果基本正常。影响血小板功能的药物（单独使用阿司匹林或与双嘧达莫联合使用）似乎可降低实验室异常的程度，表明该药物在这种疾病中可能具有临床应用价值。
