Haematological determinants of cardiac involvement in adults with sickle cell disease
Haematological determinants of cardiac involvement in adults with sickle cell disease
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DOI：
10.1093/eurheartj/ehv555
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发表时间：
2016-04-07
影响因子：
39.3
通讯作者：
Bartolucci, Pablo
中科院分区：
文献类型：
--
作者：
Damy, Thibaud;Bodez, Diane;Bartolucci, Pablo
目的镰状细胞病（SCD）常见心脏受累。需要进行研究以确定这种参与的血液学决定因素和预后标志物。该研究的目的是确定血液学因素与心脏受累的SCD和他们的预后的影响。方法和结果这纵向观察研究进行了1780 SCD患者SS或S-β（0）-地中海贫血提到我们的中心。600五十六例符合我们的入选标准（分别有35、78、23、8.5和17%的患者血液检查和超声心动图的可用性> 4 L/min/m2，LV射血分数= 2.5 m/s。与其他患者相比，左室舒张末期内径指数（LVEDDind）和左房内径指数（LADind）第四个四分位数（Q4）和高CI患者的Hb、%胎儿Hb（HbF）和红细胞（RBC）计数显著降低;乳酸脱氢酶、胆红素和%致密RBC显著升高。Q4 LVEDDind和LADind的独立血液学决定因素为低RBC计数和% HbF;高密度RBC %与LADind相关。低% HbF和RBC计数与高CI相关。高密度红细胞百分比或无α-地中海贫血基因缺失与贫血和心脏扩张的严重程度更高以及CI更高相关。在中位随访48（32-59）个月期间，50例（7.6%）患者死亡。三尖瓣返流速度>= 2.5 m/s是死亡率的预测因子。当左心室射血分数= 2.5和LV功能障碍（即使是轻度）预测死亡率时，死亡风险增加4倍。
Aims Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis.Methods and results This longitudinal observational study was performed on 1780 SCD patients with SS or S-beta(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained 4 L/min/m(2), LV ejection fraction = 2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and % dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and % HbF; high % dense RBCs were associated with LADind. Low % HbF and RBC count were associated with high CI. High % dense RBCs or no alpha-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity >= 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction = 2.5 and LV dysfunction (even mild) predict mortality.