Abstract
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.
Highlights
We aimed to investigate the pulmonary function abnormalities, and their association with pulmonary hypertension (PH) in hemoglobin E/β-Thal patients stratified by their splenic status
The final study group consisted of 25 patients, whom were further classified into 2 groups, i.e., splenectomized patients (SP) and non-splenectomized patients (NSP)
Of the 13 SP, the patterns of spirometric abnormalities were defined as having restrictive impairment (RI) in 11 (84%) patients; 2 of 11 patients having RI combined with small airway involvement
Summary
Previous studies have reported pulmonary dysfunction in patients with β-thalassemia (Thal); mostly with restrictive impairment (RI) in patients being transfusion-dependent[1,2,3,4,5,6,7,8,9,10,11] and in non-transfusion dependentPrapaporn Pornsuriyasak,[1] Kulanee Vongvivat,[1] Khanchit Likittanasombat,[2] Thitiporn Suwatanapongched,[3] Vichai Atichartakarn[4] ment of diffusion capacity,[16,17] arterial hypoxemia,[16,18] and pulmonary hypertension (PH).[19,20,21] Blood transfusion and iron chelation therapy for thalassemia disease in Thailand are usually not as intensive as in Western countries due to limited resources.
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