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2017 ; 31
(4
): 213-223
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English Wikipedia
Complementopathies
#MMPMID28215731
Baines AC
; Brodsky RA
Blood Rev
2017[Jul]; 31
(4
): 213-223
PMID28215731
show ga
The complement system is an essential part of the innate immune system that
requires careful regulation to ensure responses are appropriately directed
against harmful pathogens, while preventing collateral damage to normal host
cells and tissues. While deficiency in some components of the complement pathway
is associated with increased susceptibility to certain infections, it has also
become clear that inappropriate activation of complement is an important
contributor to human disease. A number of hematologic disorders are driven by
complement, and these disorders may be termed "complementopathies". This includes
paroxysmal nocturnal hemoglobinuria (PNH), atypical hemolytic uremic syndrome
(aHUS), cold agglutinin disease (CAD) and other related disorders, which will be
the focus of this review. A better understanding of the central role of the
complement system in the pathophysiology of these disorders may allow for
application of therapies directed at blocking the complement cascade.