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Review
. 2017:1013:59-87.
doi: 10.1007/978-1-4939-7299-9_3.

Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease

Satheesh Chonat  1 , Charles T Quinn  2
Affiliations
Review

Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease

Satheesh Chonat et al. Adv Exp Med Biol. 2017.

Abstract

Thalassemia and sickle cell disease (SCD) are disorders of hemoglobin that affect millions of people worldwide. The carrier states for these diseases arose as common, balanced polymorphisms during human history because they afforded protection against severe forms of malaria. These complex, multisystem diseases are reviewed here with a focus on current standards of clinical management and recent research findings. The importance of a comprehensive, multidisciplinary and lifelong system of care is also emphasized.

Keywords: Clinical care; Outcome; Sickle cell disease; Standard care; Thalassaemia; Thalassemia.

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Figures

Fig. 3.1
Fig. 3.1
Improving survival in thalassemia major. Complication-free survival for patients with thalassemia major cared for in Italy is shown by birth cohort (1970–74, 1975–79, 1980–84, 1985–97). From: Borgna-Pignatti C, et al. Ann N Y Acad Sci. 2005;1054(1):40–7. Reprinted with permission from John Wiley and Sons
Fig. 3.2
Fig. 3.2
Improving survival in sickle cell disease (SCD). Overall survival curves for patients with sickle cell anemia (Hb SS) and sickle-β0-thalassemia spanning by years of birth are shown for large SCD cohorts in the US, UK and Jamaica. Figure adapted from: Quinn et al. Blood. 2010;115:3447–3452
See this image and copyright information in PMC

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