Sickle Cell Anemia
Scott W. Roberts
Abstract
Scott W. Roberts
Abstract
Sickle cell hemoglobinopathy is the most common inherited disease in the world. The sickling deformation and lysis result in vaso-occlusive (VOC) events and hemolytic anemia. VOC events can be widely varied and include local infarction of all organ systems; examples include acute chest syndrome and pulmonary embolism. Obstetric management involves using multidisciplinary care including hematologists and often subspecialty doctors focused on particular morbidities in the sickle cell patient. Sickle cell pathology can involve other hemoglobinopathy such as β-thalassemia. It is important that the particular genotype of sickle cell disease (e.g. S/β 0 thalassemia) is identified in each patient. This involves electrophoresis and genetic analysis in groups at high risk for hemoglobinopathy.
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Sickle cell hemoglobinopathy is the most common inherited disease in the world. The sickling deformation and lysis result in vaso-occlusive (VOC) events and hemolytic anemia. VOC events can be widely varied and include local infarction of all organ systems; examples include acute chest syndrome and pulmonary embolism. Obstetric management involves using multidisciplinary care including hematologists and often subspecialty doctors focused on particular morbidities in the sickle cell patient. Sickle cell pathology can involve other hemoglobinopathy such as β-thalassemia. It is important that the particular genotype of sickle cell disease (e.g. S/β 0 thalassemia) is identified in each patient. This involves electrophoresis and genetic analysis in groups at high risk for hemoglobinopathy.
Key concepts: Hemoglobinopathy, Medicine, Sickle cell anemia, Thalassemia, Disease, Hemoglobin electrophoresis, Acute chest syndrome, Pediatrics