Complement biology for hematologists
Anna Duval, Véronique Frémeaux‐Bacchi
Abstract
The complement system is part of the innate immunity. An increased activation or a loss of the regulation of this fine-tuned cascade is involved in a variety of hematological diseases. During the last decade, anti-C5 therapies have revolutionized the management and prognosis of paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic and uremic syndrome (aHUS). The availability of a rapidly growing number of innovative complement inhibitors has opened new therapeutic perspectives for several other hematological disorders in which the complement is involved at different degrees. This review focuses on complement biology and its mechanisms of activation in hematological diseases.
Topics & Concepts
Paroxysmal nocturnal hemoglobinuriaComplement systemComplement (music)ImmunologyHematological disordersAtypical hemolytic uremic syndromeEculizumabMedicineAlternative complement pathwayBiologyImmune systemGeneticsPhenotypeComplementationGeneComplement system in diseasesRenal Diseases and GlomerulopathiesBlood groups and transfusion