Douglas Katz;
New York University Long Island School of Medicine
Mariam Moshiri;
University of Washington Medical Center
John Pellerito;
Zucker School of Medicine at Hofstra/Northwell Health System
Margarita Revzin;
Yale School of Medicine
Background
Sickle cell disorder (SCD) refers to a spectrum of hematologic disorders that cause a characteristic clinical syndrome affecting the entire body. It is the most prevalent monogenetic hemoglobinopathy worldwide with a wide range of focal and systemic expressions. Hemoglobin gene mutation leads to the formation of abnormal sickle-shaped red blood cells that cause vascular occlusion, resulting in tissue and organ ischemia and infarction. Recurrent episodes of acute illness lead to progressive multisystem organ damage and dysfunction. Vasoocclusion, hemolysis, and infection as a result of functional asplenia are at the heart of the disease manifestation.
Educational Goals / Teaching Points
Describe the pathophysiology of hemoglobinopathies and red blood cells injury in SCD. Define the imaging features of the common manifestations of SCD. Review the role of imaging in the diagnosis and evaluation of suspected complications of SCD
Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
We aim to familiarize the radiologist with the SCD spectrum, emphasizing the detection and evaluation of manifestations. The topics addressed include: a) pathophysiology of the disease; b) SCD placement among the hemoglobinopathies; c) clinical presentation of SCD; d) the role of imaging in the evaluation and diagnosis of patients with SCD; e) imaging features associated with common and uncommon sequelae of SCD; and f) a brief overview of management and treatment of patients with SCD.
Conclusion
Imaging plays an essential role in the diagnosis and management of SCD related complications. A thorough understanding of key imaging findings of SCD complications is crucial to timely recognition and accurate diagnosis.
