Infections in thalassemia and hemoglobinopathies: focus on therapy-related complications - PubMed (original) (raw)

Bianca Maria Ricerca et al. Mediterr J Hematol Infect Dis. 2009.

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.

PubMed Disclaimer

Figures

Figure 1.

Figure 1.

Risks related to iron overload.

Similar articles

Cited by

References

    1. Borgna-Pignatti C, Rugolotto S, De Stefano P, et al. Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica. 2004;89:1187–1193. - PubMed
    1. Ladis V, Chouliaras G, Bedousi H, et al. Longitudinal study of survival and causes of death in patients with thalassemia major in Greece. Ann N Y Acad Sci. 2005;1054:445–50. - PubMed
    1. Chern JP, Su S, Lin KH, et al. Survival, mortality, and complications in patients with beta-thalassemia major in northern Taiwan. Pediatr Blood Cancer. 2007;48:550–554. - PubMed
    1. Wanachiwanawin W. Infections in E-beta thalassemia. Pediatr Hematol Oncol. 2000;22(6):581–7. - PubMed
    1. Manci EA, Culberson DE, Yang YM, et al. Causes of death in sickle cell disease: an autopsy study. Br J Haematol. 2003;123(2):359–365. - PubMed

LinkOut - more resources