Abstract Background Prevalence of hereditary blood diseases such as sickle cell anemia, sickle thalassemia and thalassemia major are high in Khuzestan province. Sickle cell anemia and beta-thalassemia are predominantly common in Iranian Arabs. Pulmonary complications account for a large proportion of morbidity and mortality in patients with and sickle cell disease. Periodic lung function assessment is recommended to provide a diagnostic clue criterion for physicians. Objective The purpose of this study is to assess the spirometry parameters in patients with sickle beta thalassemia and sickle cell disease in south west Iran. Materials and Methods Over three months, a total of 35 patients participate in this cross sectional study. Spirometry test was performed on 21 patients with sickle cell disease (12 male, 9 female) and 14 patients with sickle beta thalassemia (7 male, 7 female) aged 6to35 years old. Normal people were matched according to age and sex and were tested as control. Results Eighty six percent of sickle cell disease and 57 percent of sickle beta thalassemia had restrictive pattern of lung disease. Forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) correlated positively with Hb F level and negatively with Hb S level (P-value<0.001) in sickle cell disease patients (P-value<0.001). Serum ferritin level had a negative effect on FEV1 and FVC in sickle beta thalassemia patients. Conclusion Periodic lung function tests are a useful monitoring test to provide a clinical evaluation profile and have positive correlation with Hb F level.

Keywords: Anemia, Sickle Cell, beta-Thalassemia, Spirometry

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