Title:Pulmonary Disease in Beta-Thalassemia
Volume: 12
Issue: 4
Author(s): Demetrios S. Theodoropoulos and Maria S. Theodoropoulou
Affiliation:
Keywords:
Beta-thalassemia, pulmonary, tranfusion.
Abstract: The care of beta-thalassemia has improved dramatically over the last few decades mostly
because of: i) improved blood transfusion regimens; ii) wide-spread use of deferoxamine; iii) surgical
advances; and iv) allogeneic hematopoietic stem cell transplantation. Advanced healthcare standards,
improved life expectancy, and complications specific to beta-thalassemia necessitate a renewed
approach to the pulmonary issues of this disease.
While transfusion-related infections are expected, and Streptococcal, Staphylococcal, E. coli and
Salmonella infections are common, a number of special problems are also reported. Mycoplasma
pneumoniae infections associated with hemolytic anemia, Pneumocystis carinii pneumonia and
necrotizing pneumonia caused by Klebsiella pneumoniae are indicative of the special susceptibility to
infections in beta-thalassemia. The issue of immune function in the (non-transplanted) patient with
thalassemia is brought up. Conditions associated with chronic airway inflammation, such as plastic
bronchitis and allergic bronchopulmonary mycosis, are discussed as well as life-threatening
complications.
In an attempt to organize the diagnostic approach to pulmonary disease in beta-thalassemia, four
working entities are delineated, which, almost invariably, tend to overlap and aggravate each other: i)
disorders inherent to the beta-thalassemia hemoglobinopathy; ii) chronic lung disease and its
complications; iii) immunological corollaries of the therapy of beta-thalassemia, i.e. chronic transfusion,
splenectomy and bone marrow transplantation; and iv) airway inflammation.
