Sickle Cell Anemia

1. Front Matter

   Pages i-xv

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2. Hemoglobin: Structure, Synthesis and Oxygen Transport

   • Susan E. Jorge, Daniela M. Ribeiro, Magnun N. N. Santos, Maria de Fátima Sonati

   Pages 1-22

3. Sickle Cell Anemia: History and Epidemiology

   • Frédéric B. Piel, Thomas N. Williams

   Pages 23-47

4. Overview of Sickle Cell Anemia Pathophysiology

   • Martin H. Steinberg

   Pages 49-73

5. Red Blood Cells and the Vaso-Occlusive Process

   • Nancy J. Wandersee, Cheryl A. Hillery

   Pages 75-90

6. Leukocytes in the Vaso-Occlusive Process

   • Dachuan Zhang, Paul S. Frenette

   Pages 91-107

7. Hypercoagulability and Sickle Cell Disease

   • Marina Pereira Colella, Erich Vinicius de Paula, Margareth Castro Ozelo, Fabiola Traina

   Pages 109-127

8. Cardiovascular Adaptations to Anemia and the Vascular Endothelium in Sickle Cell Disease Pathophysiology

   • Lydia H. Pecker, Hans C. Ackerman

   Pages 129-175

9. Inflammation and Sickle Cell Anemia

   • Camila Bononi de Almeida, Gregory J. Kato, Nicola Conran

   Pages 177-211

10. Clinical Manifestations of Sickle Cell Anemia: Infants and Children

    • Robert Sheppard Nickel, Lewis L. Hsu

    Pages 213-229

11. Treatment of Childhood Sickle Cell Disease

    • Rouba Abdennour, Miguel R. Abboud

    Pages 231-267

12. Priapism in Sickle Cell Disease: New Aspects of Pathophysiology

    • Mário A. Claudino, Carla F. Franco Penteado, Kleber Yotsumoto Fertrin

    Pages 269-283

13. Clinical Manifestations and Treatment of Adult Sickle Cell Disease

    • Fernando Ferreira Costa, Kleber Yotsumoto Fertrin

    Pages 285-318

14. Hemoglobin Sβ Thalassemia, SC Disease and SD Disease: Clinical and Laboratorial Aspects

    • Sara T. Olalla Saad, Simone O. Gilli

    Pages 319-337

15. Sickle Cell Disease in Africa and the Arabian Peninsula: Current Management and Challenges

    • Adekunle Adekile, Julie Makani

    Pages 339-370

16. Genetic Factors Modifying Sickle Cell Disease Severity

    • Kate Gardner, Swee Lay Thein

    Pages 371-397

17. Future Perspectives for the Treatment of Sickle Cell Anemia

    • Kerri Nottage, Jeremie Estepp, Jane Hankins

    Pages 399-429

18. Back Matter

    Pages 431-435

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Although sickle cell anemia was the first molecular disease to be identified, its complex and fascinating pathophysiology is still not fully understood. A single mutation in the beta-globin gene incurs numerous molecular and cellular mechanisms that contribute to the plethora of symptoms associated with the disease. Our knowledge regarding sickle cell disease mechanisms, while still not complete, has broadened considerably over the last decades. Sickle Cell Anemia: From Basic Science to Clinical Practice aims to provide an update on our current understanding of the disease’s pathophysiology and use this information as a basis to discuss its manifestations in childhood and adulthood. Current therapies and prospects for the development of new approaches for the management of the disease are also covered.

• Hematology
• Hemoglobin
• Hereditary Disease
• Red Cells
• Sickle Cell Anemia

• Centro de Hematologia e Hemoterapia, Unicamp, Campinas, Brazil

  Fernando Ferreira Costa, Nicola Conran

Fernando F. Costa - graduated (1974) and obtained both his MSc (1979) and PhD (1981) degrees from the Ribeirão Preto School of Medicine of the University of São Paulo, where he also served as a faculty member (1985-1989). After completing a postdoctoral fellowship at the Yale School of Medicine (1987-1989), he joined the Department of Internal Medicine of the School of Medical Sciences of the University of Campinas (1990) and became a Full Professor of Hematology and Hemotherapy (1996). Member of several national and international academic societies, Professor Costa has already published 260 papers in peer-reviewed journals and supervised 29 doctoral theses. He received a decoration from the Government of Brazil (2008) and has been awarded a great number of scientific prizes, including one from the Government of the State of São Paulo (2000). At the University of Campinas, Professor Costa served as Dean of the School of Medical Sciences (1994-1998), Director of the Hematology and Hemotherapy Center (1998-2002), Vice-President for Research (2002-2005) and General Coordinator of the University (2005-2009). He was the president of the University of Campinas from April 2009 to April 2013.
Nicola Conran - Research scientist at the University of Campinas, Brazil. Graduated in Biochemistry (University of Birmingham, UK); PhD, University of Nottingham, UK.

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