The most comprehensive, current sickle cell disease resource—for both clinicians and researchers
The first and only resource of its kind, Sickle Cell Disease examines this blood disorder through both clinical and research lenses. More than 80 dedicated experts in the field present their combined clinical knowledge of basic mechanisms, screening, diagnosis, management, and treatment of myriad complex complications of a single base point mutation in the human genome. Case studies with “How I Treat” authoritative insights provide overviews of common and rare complications, and Key Facts offer at-a-glance high-yield information.
Filled with clinical photos, illustrations, numerous original diagrams, and with free updates available online, this unmatched resource covers:
Mechanisms of sickle cell disease
Historic and current research approaches
The latest work in gene therapy and editing
Guidelines for patient care, diagnosis, unique cases, and therapies
Rare and common complications, including domestic and internationally relevant topics
Psychosocial and supportive care
The newest standards of therapy and future treatment options in children and adults
Cardiopulmonary complications
Table of Contents
I. Basic Mechanisms of Disease:
1. Overview: Carlo Brugnara, Betty Pace
2. Genetic basis of SCD: Martin Steinberg, SweeLay Thein
3. Hemoglobin S polymerization: Daniel Kim-Shapiro, Alan Schechter, and Constance Noguchi
4. Adhesion and vaso-occlusion - the role of leukocytes: Paul Frenette,
5. Hemolysis and endothelial dysfunction: Mark Gladwin, Gregory Kato
6. Sterile inflammation and the inflammasome: Prithu Sundd, Solomon Ofori-Acquah
7. IIschemia-reperfusion injury, inflammation and oxidative stress: Robert Hebbel, John Belcher, Gregory Vercellotti
8. Mouse models of SCD and mechanisms of pain:Cheryl Hillery, Kalpna Gupta, Lewis Hsu
9. Fetal hemoglobin induction - past, present and future: Abdullah Kutlar, Griff Rodgers
II. Clinical Complications of Sickle Cell Disease:
Overview: Alexis Thompson, Ken Ataga
10. Stroke and cognitive dysfunction: Fenella Kirkham, Michael DeBaun, Robert Adams
11. Pediatric complications and newborn screening: John J. Strouse, James Casella, Nancy Green
12. Airway hyperreactivity and asthma: Claudia Morris, Liz Klings
13. Acute chest syndrome:Elliott Vichinsky, Armand Mekontso-Dessap
14. Pulmonary hypertension (PH) and critical illness: Roberto Machado, Mark Gladwin
15. Heart disease: Vandana Sachdev, Punam Malik,John Wood
16. Renal: Victor Gordeuk,Santosh Saraf, Jane Little
17. Sickle cell trait: Philippe Connes, Rakhi Naik, Hyacinth Hyacinth
18. Thrombophilia in SCD: Nigel Key, Rafal Pawlinski, Enrico Novelli
19. Acute and chronic pain: Wally Smith, Samir Ballas
20. “Orphan” complications - leg ulcers and priapism: Caterina Minniti, Arthur Burnett
21. Ocular complications: : Adrienne Scott, Morton Goldberg
22. Rare presentations and emerging complications: Frédéric Galacteros, Oswaldo Castro, Kathryn Hassell
23. Globalburden of disease: new frontiers in SCD research and care: Kwaku Ohene-Frempong, Julie Makani, Fernando Costa, Graham Serjeant,Yazdi Italia
III. EmergingTherapeutics:
Overview: David Rees, Sophie Lanzkron
24. Transfusion medicine: Anoosha Habibi, Darrell Triulzi, Miguel Abboud, Sally Campbell Lee
25. Clinical trials - state of the art and lessons learned: Ken Ataga, Laura De Castro, Marilyn Telen
26. Stem cell transplantation: John Tisdale,Courtney Fitzhugh, Mark Walters, Lakshmanan Krishnamurti
27. Emergency care – challenges and opportunities: ClaudiaMorris
28. The psychosocial burden in SCD: MarciaTreadwell, Allison King, Julie Panepinto, Charles Jonaissant
29. Hydroxyurea and the dawn of combination therapy in SCD: Winfred Wang, Russell Ware
30. The HOLY GRAIL - Gene editing and Therapy: TimTownes, Marina Cavazzana-Calvo
Additional authors:
Joshua Field (chapter assignment TBD)
