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The effect of splenectomy on pulmonary function test (PFT) and pulmonary hypertension (PH) in thalassemia remains unclear. We aimed to investigate PFT and their association with PH in patients with hemoglobin E/β-thalassemia stratified by their splenic status. Thirteen splenectomized patients (SP) and 12 non-splenectomized patients (NSP) were compared regarding to the PFT abnormalities and PH (mean pulmonary artery pressure from right-heart catheterization ≥25 mmHg or estimated systolic pulmonary artery pressure from echocardiography ≥40 mmHg). Eleven (84%) SP and 9 (75%) NSP had restrictive impairment (RI). Of these, more patients having severe RI in SP than in NSP (8 vs 2, P=0.035). FVC and PaO2 were lower in SP than in NSP (66±15% vs 77±12%, P=0.043, and 79.38±1.6 mmHg vs 98.83±6.2 mmHg, P<0.001, respectively). Residual volume was higher in SP than in NSP (78±17% vs 64±15%, P=0.036). Seven (54%) SP who developed PH had a longer time interval between splenectomy and the onset of PH than those who did not (17±4.9 years vs 9.8±6.1 years, P=0.04). In conclusion, greater severity of extrapulmonary restrictive impairment and hypoxemia were more common in SP. These patients developed PH as a late complication unrelated to hypoxemia and PFT parameters. 　　因脾脏切除而对肺功能测试(PFT)以及地中海贫血症中肺动脉高压 (PH)情况造成的影响尚不明确。我们旨在通过对血红蛋白E/β-地中海贫血症的患者进行脾脏位置的分级来探查肺功能测试(PFT)和肺动脉高压 (PH)之间的相关性。十三位脾切除患者(SP)和十二位未切除脾脏的患者(NSP)参与了有关肺功能测试(PFT)和肺动脉高压 (PH)(肺动脉高压是指从右心房导管术测量的肺动脉平均压力≥25 mmHg或者由超声心动图所估计的收缩期的肺动脉平均压力≥40 mmHg)的对比检查。十一位脾切除患者(SP)(84%)和九位未切除脾脏的患者(NSP)(75%)显现出了限制性的障碍 (RI)。毫无疑问脾切除患者(SP)对于未切除脾脏的患者(NSP)表现出了更严重的限制性障碍(RI)(8 比2, P=0.035)。脾切除患者(SP)比未切除脾脏的患者(NSP)表现出更低的肺活量最大值及动脉氧分压值(相对应地66±15% 比77±12%, P=0.043, 79.38±1.6 mmHg 比98.83±6.2 mmHg, P<0.001)。对于余气量SP高于NSP(对应的78±17%比 64±15%, P=0.036)。七位(54%)SP并有 PH症状的患者显现出在首度患有肺动脉高压PH至脾切除手术之间的更长的时间间隔。综上所述在SP群体中显现出了更严重的肺外限制性障碍以及低血氧症状。对于这类患者患上肺动脉高压作为晚期并发症与他们在低血氧和肺功能测试的结果之间并无相关性。</p

thalassemia reports

Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension