The improved survival of patients with Thalassemia Major (TM) has allowed new complications to develop in the adult patients. Pulmonary dysfunction is one of the least understood, previously unrecognized, complications of TM. Aim: to define the spirometric pattern and lung damage in 35 TM patients (18 M, 17 F, age 18-44 years). All were regularly transfused (pre transfusion hemoglobin level 9+/-0.4 g/dl) and chelated (25 with deferoxamine, 5 with deferiprone and 5 with both chelators). Their serum ferritin level was 1071+/-628 ng/ml All patients had normal cardiac ejection fraction. 9 patients were active smokers while 9 had been smokers in the past. Four had a mild allergic asthma under good clinical control, 1 had a mild pulmonary hypertension and 1 had mediastinal hematopoietic tissue. Respiratory function tests (pulmonary volumes and flows and TLCO) were performed before transfusion. Results: 1 patient (the one with mediastinal masses) had an obstructive pattern (FEV1/VC 53% FEV1 31%). Thirteen patients (37%) had a restrictive pattern (mean FEV1/VC 96%+/-4% ,TLC 73%+/-1.8%). A decreased TLCO (mean 53%+/-1.5%) that can be indicative of parenchymal disease was present in 29 patients (85.8%) even after correction for hemoglobin levels. No correlation was found with sex, age, smoking, asthma diagnosis, ferritin levels, chelation regimen. The restrictive lung function abnormality with reduced diffusion capacity found in our patients is suggestive of interstitial lung disease secondary to TM. We were unable to correlate the restrictive and interstitial lung disease to iron loading nor to chelation strategies. Further studies are needed to determine whether different chelation regimens have different impact on pulmonary function.
Pulmonary Dysfunction in Thalassemic Patients
GENNARI, Alessandra;BELLAGAMBA, Maria Paola;FERRARO, Andrea;COGO, Annaluisa;BORGNA, Caterina
2006
Abstract
The improved survival of patients with Thalassemia Major (TM) has allowed new complications to develop in the adult patients. Pulmonary dysfunction is one of the least understood, previously unrecognized, complications of TM. Aim: to define the spirometric pattern and lung damage in 35 TM patients (18 M, 17 F, age 18-44 years). All were regularly transfused (pre transfusion hemoglobin level 9+/-0.4 g/dl) and chelated (25 with deferoxamine, 5 with deferiprone and 5 with both chelators). Their serum ferritin level was 1071+/-628 ng/ml All patients had normal cardiac ejection fraction. 9 patients were active smokers while 9 had been smokers in the past. Four had a mild allergic asthma under good clinical control, 1 had a mild pulmonary hypertension and 1 had mediastinal hematopoietic tissue. Respiratory function tests (pulmonary volumes and flows and TLCO) were performed before transfusion. Results: 1 patient (the one with mediastinal masses) had an obstructive pattern (FEV1/VC 53% FEV1 31%). Thirteen patients (37%) had a restrictive pattern (mean FEV1/VC 96%+/-4% ,TLC 73%+/-1.8%). A decreased TLCO (mean 53%+/-1.5%) that can be indicative of parenchymal disease was present in 29 patients (85.8%) even after correction for hemoglobin levels. No correlation was found with sex, age, smoking, asthma diagnosis, ferritin levels, chelation regimen. The restrictive lung function abnormality with reduced diffusion capacity found in our patients is suggestive of interstitial lung disease secondary to TM. We were unable to correlate the restrictive and interstitial lung disease to iron loading nor to chelation strategies. Further studies are needed to determine whether different chelation regimens have different impact on pulmonary function.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
