Tsitsikas Dimitris A, Orebayo Funmilayo, Agapidou Alexandra, Amos Roger J
Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London, UK.
Transfus Apher Sci. 2017 Oct;56(5):713-716. doi: 10.1016/j.transci.2017.08.001. Epub 2017 Aug 18.
Two main sub-phenotypes have been described in sickle cell disease: one with higher baseline haemoglobin and a higher rate of painful crises and one with lower baseline haemoglobin, increased markers of haemolysis and a higher incidence of pulmonary hypertension, priapism and leg ulcers. We compared the patterns of response to regular automated red cell exchange transfusion over a five-year period of 21 patients with recurrent painful crises from the first group and 3 patients with pulmonary hypertension and 5 with recurrent severe stuttering priapism form the second and found them to be distinctly different. Response for pain is slow and increases gradually over years. The most pronounced clinical benefit and the one that appears first is a reduction in the severity rather than the frequency of painful crises. In contrast to the slow and gradual response we see for pain, response of patients with pulmonary hypertension and priapism is immediate with significant clinical improvement even after the first transfusion. The response appears to be directly correlated to the HbS level as the symptoms of both conditions invariably recur rapidly when transfusions are delayed or discontinued but resolve again once they are re-instituted.
一种基线血红蛋白水平较高，疼痛性危象发生率较高；另一种基线血红蛋白水平较低，溶血标志物升高，肺动脉高压、阴茎异常勃起和腿部溃疡的发生率较高。我们比较了第一组21例复发性疼痛性危象患者、第二组3例肺动脉高压患者和5例复发性严重间歇性阴茎异常勃起患者在五年期间对定期自动红细胞置换输血的反应模式，发现它们明显不同。疼痛的反应缓慢，且多年来逐渐增强。最显著的临床益处且最先出现的是疼痛性危象严重程度的降低而非发作频率的降低。与我们看到的疼痛的缓慢渐进反应相反，肺动脉高压和阴茎异常勃起患者的反应是即时的，即使在首次输血后临床症状也有显著改善。这种反应似乎与血红蛋白S（HbS）水平直接相关，因为当输血延迟或中断时，这两种情况的症状总是会迅速复发，但一旦重新开始输血，症状又会再次缓解。
