Affiliations: [a] Department of Medicine, Division of Pulmonary, Critical Care, Sleep and Allergy, University of Illinois at Chicago, Chicago, IL, USA
| [b] Department of Medicine, Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, Indiana University, Indianapolis, IN, USA
Correspondence:
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Corresponding author: Roberto F. Machado, MD, Division of Pulmonary, Critical Care, Sleep, and Occupational Medicine, Indiana University, Department of Medicine, Room C400, Walther Hall, R3 980W. Walnut Street, Indianapolis, IN 46202, USA. Tel./Fax: +1 317 278 0047; E-mail: [email protected].
Abstract: Sickle cell disease (SCD) is a monogenetic disorder caused by a mutation in the β-globin gene HBB leading to polymerization of red blood cells causing damage to cell membranes, increasing its rigidity and intravascular hemolysis. Multiple lines of evidence suggest that SCD can be viewed as pan-vasculopathy associated with multiple mechanisms but driven by hemoglobin S polymerization. Here we review the pathophysiology, clinical manifestations and management strategies for cerebrovascular disease, pulmonary hypertension and renal disease associated with SCD. These “vascular phenotypes” reflect the systemic nature of the complications of SCD and are a major threat to the well-being of patients with the disorder.
