title: Crises in Sickle Cell Disease.
abstract: In spite of significant strides in the treatment of sickle cell disease (SCD), SCD crises are still responsible for high morbidity and early mortality. While most patients initially seek care in the acute setting for a seemingly uncomplicated pain episode (pain crisis or vaso-occlusive crisis), this initial event is the primary risk factor for potentially life-threatening complications. The pathophysiological basis of these illnesses is end-organ ischemia and infarction combined with the downstream effects of hemolysis that results from red blood cell sickling. These pathological changes can occur acutely and lead to a dramatic clinical presentation, but are frequently superimposed over a milieu of chronic vasculopathy, immune dysregulation, and decreased functional reserve. In the lungs, acute chest syndrome is a particularly ominous lung injury syndrome with a complex pathogenesis and potentially devastating sequelae, but all organ systems can be affected. It is, therefore, critical to understand the SCD patients' susceptibility to acute complications and their risk factors so that they can be recognized promptly and managed effectively. Blood transfusions remain the mainstay of therapy for all severe acute crises. Recommendations and indications for the safest and most efficient implementation of transfusion strategies in the critical care setting are therefore presented and discussed, together with their pitfalls and potential future therapeutic alternatives. In particular, the importance of extended phenotypic red blood cell matching cannot be overemphasized, due to the high prevalence of severe complications from red cell alloimmunization in SCD.
authors.affiliation: Pittsburgh Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA; Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA.
authors.forename: Enrico M
authors.initials: EM
authors.lastname: Novelli
authors.affiliation: Pittsburgh Heart, Lung, and Blood Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, PA; Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA; Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA. Electronic address: gladwinmt@upmc.edu.
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
mesh_terms: D056586:Acute Chest Syndrome / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D000741:Anemia, Aplastic / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D000755:Anemia, Sickle Cell / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D001788:Blood Grouping and Crossmatching / Q000379:methods; D018450:Disease Progression; D017707:Erythrocyte Transfusion / Q000379:methods; D005078:Exchange Transfusion, Whole Blood / Q000379:methods; D006801:Humans; D009102:Multiple Organ Failure / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D010102:Oxygen Inhalation Therapy; D010146:Pain / Q000209:etiology; D059408:Pain Management; D054038:Posterior Leukoencephalopathy Syndrome; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D020521:Stroke / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*
chemical_list: D000900:Anti-Bacterial Agents
keywords: acute chest syndrome; red blood cells; sickle cell disease; transfusion
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