الفهرس 
Introduction.Only 14 pages are availabe for public view
 |  | from | 130 |  |  |
| | | from | 130 | | |
Abstract
Complement is an important component of the innate immune system that is
crucial for defense from microbial infections and for clearance of immune
complexes and injured cells.
The complement system consists of a number of small proteins found in the
blood, generally synthesized by the liver, and normally circulating as inactive
precursors . When stimulated by one of several triggers, proteases in the system
cleave specific proteins to release cytokines and initiate an amplifying cascade of
further cleavages. The end-result of this activation cascade is massive
amplification of the response and activation of the cell-killing membrane attack
complex.
Normaly, complement is tightly controlled by a number of fluid-phase and
cell surface proteins to avoid injury to autologous tissues.
Eculizumab is a humanized anti-C5 monoclonal antibody that blocks the
formation of C5a and the terminal MAC of complement activation .
Complement induced blood diseases may be caused by deposition of
circulating active complement fragments on surfaces of different blood cells, but
complement locally produced and activated in the kidney as in atypical haemolytic
uremic syndrome also may have a role. Many blood disorders have been linked to
abnormal complement activation or dysregulation, including cold agglutinin
disease, antiphosphlipid syndrome ,heridetary angioedema and paroxysmal
nocturnal haemoglobinurea.