F. Santamaria et al., THE EFFECT OF TRANSFUSION ON PULMONARY-FUNCTION IN PATIENTS WITH THALASSEMIA MAJOR, Pediatric pulmonology, 18(3), 1994, pp. 139-143
Pulmonary involvement has been documented in thalassemia major (TM). W
e studied 12 patients with TM before and 24 hr after transfusion to ev
aluate the effect of transfusion on baseline lung function. Personal a
nd family histories of respiratory illnesses were obtained by a questi
onnaire. Spirometry and carbon monoxide diffusion capacity (KCO) measu
rements were made. Blood gases (P-O2 and S-O2) were determined on arte
rialized samples. Baseline expiratory volumes and flows were within no
rmal range in all patients. Transfusion resulted in a significant redu
ction of forced expiratory volume in 1 sec (FEV(1)) and forced expirat
ory flow between 25 and 75% vital capacity (FEF(25-75%)). In two subgr
oups of patients identified by the questionnaire, those with no histor
y of airway disease had normal baseline flows and no posttransfusion c
hanges; those with history of airway obstruction had lower pretransfus
ion flows and significantly decreased posttransfusion FEV(1) and FEF25
-75%. The mean pretransfusion KCO value of 80% predicted for the whole
group, significantly increased after transfusion (P < 0.05). Blood ga
ses also significantly increased after transfusion (P < 0.05). When te
sted for the spirometric response to albuterol, patients with a histor
y of asthma had a slightly greater increase in FEV(1) and FEF(25-75%)
than those who had never had asthma. We conclude that in our small stu
dy group, transfusion resulted in improved gas exchange and lung perfu
sion. The effect on flow limitation evident in some patients could, in
part, be related to a preexisting bronchial hyperreactivity. Accurate
evaluation of pulmonary function and of bronchial reactivity is advis
able for patients with TM. (C) 1994 Wiley-Liss, Inc.