Ataga Kenneth I, Moore Charity G, Jones Susan, Olajide Oludamilola, Strayhorn Dell, Hinderliter Alan, Orringer Eugene P
Division of Hematology/Oncology and Comprehensive Sickle Cell Program, University of North Carolina, Chapel Hill, NC, USA.
Br J Haematol. 2006 Jul;134(1):109-15. doi: 10.1111/j.1365-2141.2006.06110.x.
Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development of PHT and the factors that affect disease progression are unknown. We observed 93 patients over a median follow-up period of 2.6 years (range 0.2-5.1 years). Data were censored at the time of death or loss to follow-up. Pulmonary hypertension was associated with an increased risk of death (relative risk, 9.24; 95% confidence interval: 1.2-73.3; P = 0.01). There was no difference in the risk of death when patients with different degrees of PHT were compared. Lactate dehydrogenase and blood urea nitrogen were significantly associated with PHT in a logistic regression model. Higher levels of fetal haemoglobin and treatment with hydroxycarbamide were observed more frequently in patients without PHT. Thirteen per cent of patients with no previous evidence of PHT developed PHT following 3 years of observation.
(1) PHT, regardless of severity, is associated with an increased risk of death in SCD patients; (2) haemolysis is strongly associated with PHT in SCD; (3) high levels of fetal haemoglobin and hydroxycarbamide therapy may decrease the occurrence of PHT; (4) screening for PHT is indicated for SCD patients in their non-crisis, steady states.
尽管肺动脉高压（PHT）是镰状细胞病（SCD）患者的常见并发症，但PHT的发展速率以及影响疾病进展的因素尚不清楚。我们对93例患者进行了中位随访期为2.6年（范围0.2 - 5.1年）的观察。数据在死亡或失访时进行截尾。肺动脉高压与死亡风险增加相关（相对风险，9.24；95%置信区间：1.2 - 73.3；P = 0.01）。比较不同程度PHT的患者时，死亡风险无差异。在逻辑回归模型中，乳酸脱氢酶和血尿素氮与PHT显著相关。在无PHT患者中，胎儿血红蛋白水平较高且接受羟基脲治疗的情况更常见。在3年观察期后，13%既往无PHT证据的患者出现了PHT。
（1）无论严重程度如何，PHT均与SCD患者死亡风险增加相关；（2）溶血与SCD中的PHT密切相关；（3）胎儿血红蛋白水平高和羟基脲治疗可能会降低PHT的发生；（4）对于处于非危象、稳定状态的SCD患者，建议筛查PHT。
