Complement biology for hematologists

Am J Hematol. 2023 May:98 Suppl 4:S5-S19. doi: 10.1002/ajh.26855.

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.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Biology
  • Complement Activation
  • Complement Inactivating Agents / therapeutic use
  • Complement System Proteins* / therapeutic use
  • Hemoglobinuria, Paroxysmal* / drug therapy
  • Humans
  • Immunity, Innate

Substances

  • Complement System Proteins
  • Complement Inactivating Agents