Abstract
The clinical approach to thalassemia and hemoglobinopathies, specifically Sickle Cell Disease (SCD), based on transfusions, iron chelation and bone marrow transplantation has ameliorated their prognosis. Nevertheless, infections still may cause serious complications in these patients. The susceptibility to infections in thalassemia and SCD arises both from a large spectrum of immunological abnormalities and from exposure to specific infectious agents. Four fundamental issues will be focused upon as central causes of immune dysfunction: the diseases themselves; iron overload, transfusion therapy and the role of the spleen. Thalassemia and SCD differ in their pathogenesis and clinical course. It will be outlined how these differences affect immune dysfunction, the risk of infections and the types of most frequent infections in each disease. Moreover, since transfusions are a fundamental tool for treating these patients, their safety is paramount in reducing the risks of infections. In recent years, careful surveillance worldwide and improvements in laboratory tests reduced greatly transfusion transmitted infections, but the problem is not completely resolved. Finally, selected topics will be discussed regarding Parvovirus B19 and transfusion transmitted infections as well as the prevention of infectious risk postsplenectomy or in presence of functional asplenia.
Highlights
Infections are a frequent complication of thalassemias and hemoglobinopathies and they can be fatal
Similar results were reported in Greece[2] and in Taiwan[3], while in E-beta thalassemia patients in Thailand, infections are the primary cause of morbidity and mortality[4]
Darbari et al[6], in 141 autopsies in Sickle Cell Disease (SCD) patients between 1976-2001, reported a lower mortality rate due to infections (18.4%) and infections were the fourth cause of death after pulmonary hypertension (PHT), the and renal failure
Summary
Infections are a frequent complication of thalassemias and hemoglobinopathies and they can be fatal. Darbari et al[6], in 141 autopsies in SCD patients between 1976-2001, reported a lower mortality rate due to infections (18.4%) and infections were the fourth cause of death after pulmonary hypertension (PHT), the and renal failure. Both of these studies were conducted in USA. Transfusion and chelation therapies represent true progress in the management of these diseases They dramatically ameliorated the prognosis of thalassemia and SCD, as epidemiological data clearly demonstrate[1,2,9]. The authors suggest that, in this model, the relationship of this alteration to IOL not caused by transfusions but results from the disease itself
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