title: Laboratory tests for disorders of complement and complement regulatory proteins.
abstract: 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.
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, 02114.
authors.forename: Angela R
authors.initials: AR
authors.lastname: Shih
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts, 02114.
authors.forename: Mandakolathur R
authors.initials: MR
authors.lastname: Murali
mesh_terms: D064790:Clinical Laboratory Services / Q000706:statistics & numerical data*; D003167:Complement Activation / Q000276:immunology*; D003165:Complement System Proteins / Q000276:immunology*; D006801:Humans
chemical_list: D003165:Complement System Proteins