Sickle cell disease--pathophysiology, clinical and diagnostic implications.
We review the current knowledge of the pathophysiology of sickle cell disease (SCD), the clinical complications and the state of the art in SCD diagnostics. Today, a flexible laboratory concept allows the fast and economic clarification of the patients' sickle cell hemoglobin (HbS) status, e.g. additional compound heterozygosities. In contrast to a well-investigated pathophysiology of the disease, factors influencing the severity of symptoms as well as some laboratory findings in SCD still lack a final explanation. In this review, we focus on red cell lysis resistance as an additional diagnostic tool in SCD. There is a need for further studies regarding lysis resistance in blood samples from patients with HbS.
Dorn-Beineke
Alexandra
A
Department of Clinical Chemistry, Faculty of Clinical Medicine, Mannheim University, Heidelberg, Heidelberg, Germany. alexandra.dorn-beineke@ikc.ma.uni-heidelberg.de
Frietsch
Thomas
T
D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology*; D004910:Erythrocyte Membrane / Q000502:physiology; D004913:Erythrocytes, Abnormal / Q000502:physiology; D006451:Hemoglobin, Sickle / Q000737:chemistry / Q000235:genetics; D006461:Hemolysis / Q000502:physiology; D006801:Humans
D006451:Hemoglobin, Sickle