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Laboratory tests for disorders of complement and complement regulatory proteins
Author(s) -
Shih Angela R.,
Murali Mandakolathur R.
Publication year - 2015
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24209
Subject(s) - complement system , alternative complement pathway , atypical hemolytic uremic syndrome , immunology , complement (music) , hereditary angioedema , complement component 5 , complement deficiency , complement component 2 , medicine , innate immune system , classical complement pathway , complement component 3 , eculizumab , complement receptor , immune system , biology , genetics , gene , complementation , phenotype
The complement pathway is a cascade of proteases that is involved in immune surveillance and innate immunity, as well as adaptive immunity. Dysfunction of the complement cascade may be mediated by aberrations in the pathways of activation, complement regulatory proteins, or complement deficiencies, and has been linked to a number of hematologic disorders, including paroxysmal noctural hemoglobinuria (PNH), hereditary angioedema (HAE), and atypical hemolytic‐uremic syndrome (aHUS). Here, current laboratory tests for disorders of the complement pathway are reviewed, and their utility and limitations in hematologic disorders and systemic diseases are discussed. Current therapeutic advances targeting the complement pathway in treatment of complement‐mediated hematologic disorders are also reviewed. Am. J. Hematol. 90:1180–1186, 2015. © 2015 Wiley Periodicals, Inc.