Evidence of lymphocyte alveolitis by bronchoalveolar lavage in thalassemicpatients with pulmonary dysfunction

Pulmonary dysfunction represents one of the least studied complications in thalassemic patients. Probably, it is due to the absence of pulmonary sympt oms. There are few works in the literature, and contradictory results have been published. The aim of this study was to define the spirometric pattern and the possible causes of lung impairment by testing bronchoalveolar lava ge (BAL) with pathological pulmonary function tests (PFTs). Furthermore, di ffusion capacity tests for carbon monoxide corrected for hemoglobin value ( Dco*) were performed. We studied 48 thalassemic patients (27 F and 21 M), w ith an age range from 8 to 23 years, divided into two groups on the basis o f PFTs results. Thus, group A was formed by 16 patients with restrictive sp irometric patterns of whom 14 had also reduced Dco* values and group B cons isted of 32 patients with normal PFTs and Dco* values. Patients of group A underwent chest high-resolution computing tomography (CHRCT) and BAL whose fluid was analyzed by microbiologic and cytological assays. A pathological CHRCT picture was present in 8 patients. Nine out of 16 patients who accept ed to undergo BAL had a chronological age greater than 17 years with a mean bone age of 13.9 years. BAL results showed lymphocyte alveolitis in 6 pati ents and a normal cytogram in 3, while alveolar iron-laden macrophages were present in 4 out of 6 patients with alveolitis and 2 out of 3 patients wit h normal cytogram. Moreover, all examined BAL fluids showed a normal CD4/CD 8 ratio, while only 2 patients showed an altered serum CD4/CD8 ratio. We de monstrated the presence of (1) lung-restrictive syndrome in 16 of the oldes t thalassemic patients; (2) lymphocyte alveolitis in 6 patients, and (3) a picture of interstitial fibrosis by CHRCT in 8 of them. All these data are suggestive of a diagnosis of interstitial lung disease secondary to thalass emia. BAL helped to identify the presence of alveolar iron-laden macrophage s that represented a local defense mechanism against free iron. This latter finding therefore might be the primary cause of the lung impairment promot ing an oxidative damage. Further studies are needed to investigate this hyp othesis and therapeutical potentials. Copyright (C) 2000 S. Karger AG. Base l.