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Pathophysiological Consequences of Hemolysis in Sickle Cell Disease (pp. 69-96) $100.00
Authors:  (Rajiv Lochan Tiwari, Gowtham Kumar Annarapu and Prasenjit Guchhait, Regional Centre for Biotechnology, Delhi NCR, India, and others)
Abstract:
Sickle cell disease (SCD) is a hemolytic disorder, caused by a single
gene mutation in the β-globin chain of hemoglobin (Hb), and is
associated with significant morbidity and mortality around the world. The
pathophysiology of SCD is very complex. Sickle cell crisis includes
several independent clinical symptoms such as hemolysis, vaso-occlusive
pain episodes, acute chest syndrome, pulmonary hypertension, priapism
and ischemic strokes. Many studies show a unique association of
intravascular hemolysis with the above clinical events in SCD. The
sickling, deformation and destruction of red blood cells (RBCs) result in
extensive release of cytoplasmic contents, especially large quantities of
Hb molecules, into the surrounding fluid. Upon release, the tetrameric Hb
molecules are converted into dimers, and also oxidized into
methemoglobin (metHb). The Hb-dimers and metHb molecules are
cleared from circulation by the haptoglobin and hemopexin mediated
pathways. Finally, the saturation of the above clearing mechanisms
results in the accumulation of excessive extracellular Hb in patients’
plasma. Extracellular Hb has many cytotoxic effects on cellular functions.
For instance, the scavenging of endogenous nitric oxide and
dysregulation of arginine metabolism by extracellular Hb is associated
with vessel constrictions, resulting in abnormalities such as pulmonary
hypertension and ischemic strokes in SCD. Besides, the oxidative effects
of Hb are associated with endothelium damage and hypercoagulable state
in patients. Our own work and that of others indicate that the decreased
proteolysis of von Willebrand factor (VWF) multimers is initiated by the
inhibitory effects of Hb. The excessive cell-free Hb in plasma could lead
to the accumulation of uncleaved VWF multimers, which are ultra-large
molecules hyper-reactive to platelets, promoting thrombotic
complications in SCD patients. A detailed insight into the pathophysiological
consequences of hemolysis has been described in this
chapter. 


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Pathophysiological Consequences of Hemolysis in Sickle Cell Disease (pp. 69-96)