TY - CHAP M1 - Book, Section TI - Chapter 433. Hemolytic Anemia A1 - Yates, Amber Mayfield A1 - Ware, Russell E. A2 - Rudolph, Colin D. A2 - Rudolph, Abraham M. A2 - Lister, George E. A2 - First, Lewis R. A2 - Gershon, Anne A. PY - 2011 T2 - Rudolph's Pediatrics, 22e AB - Red blood cells (RBCs) have a normal life span of approximately 120 days. An operational definition of hemolysis, therefore, is accelerated RBC destruction with premature removal from the circulation, which usually causes anemia. Hemolytic anemia can occur in a wide range of clinical settings featuring various etiologies; signs and symptoms reflect the location and severity of hemolysis. Intravascular hemolysis occurs when erythrocytes are destroyed in the blood vessel itself, whereas extravascular hemolysis occurs in the hepatic and splenic macrophages within the reticuloendothelial system. Intravascular hemolysis is often dramatic, with free hemoglobin released into the plasma leading to hemoglobinuria (positive blood on urine dipstick but few erythrocytes on microscopic examination). Examples of intravascular hemolysis include enzyme defects such as glucose-6-phosphate dehydrogenase (G6PD) deficiency or certain immune-mediated processes. Extravascular hemolysis usually results from more subtle RBC destruction, typically with chronic splenic enlargement and jaundice. Extravascular hemolysis is more common with RBC membrane disorders such as hereditary spherocytosis. Some forms of hemolytic anemia feature both intravascular and extravascular hemolysis. The degree of anemia depends on how rapidly the erythrocytes are being removed from circulation and how well the bone marrow compensates with increased reticulocytosis. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/04/19 UR - accesspediatrics.mhmedical.com/content.aspx?aid=7039705 ER -