TY - CHAP M1 - Book, Section TI - Rare Presentations and Emerging Complications of Sickle Cell Disease A1 - Castro, Oswaldo A1 - Hassell, Kathryn L. A2 - Gladwin, Mark T. A2 - Kato, Gregory J. A2 - Novelli, Enrico M. Y1 - 2021 N1 - T2 - Sickle Cell Disease AB - This chapter addresses clinical presentations of sickle cell disease that are uncommon or newly emerging or that pose special diagnostic and therapeutic challenges. Many of them are life threatening or can result in severe loss of organ function. Some clinical presentations also illustrate features of sickle cell disease pathophysiology that are not always recognized in the more common complications such as pain crisis and chest syndrome. There are no clinical trials and few published reviews dealing with these issues, their pathogenetic mechanisms, or their treatment. Hence, the diagnostic and management measures suggested here necessarily are based on published single reports and small case series and on clinical experience. Acute multiorgan failure syndrome is summarized first because of its life-threatening potential and also because its clinical features and treatment are similar to some of the other, less common conditions discussed. Information on drug-induced pain episodes (crises) is included primarily to promote awareness of these newly emerging and unexpected complications. The chapter ends with descriptions of how other, nonsickling, red cell disorders affect patients with sickle cell disease, with a special emphasis on the immune hemolysis that characterizes sickle-related hyperhemolysis syndrome. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - hemonc.mhmedical.com/content.aspx?aid=1179344817 ER -