Complement in hemolytic anemia

Research output: Contribution to journalArticle

Abstract

Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributable to mutations that lead to activation of the alternative pathway of complement. For optimal therapy, it is critical, but often difficult, to distinguish aHUS from other TMAs, such as thrombotic thrombocytopenic purpura; however, novel bioassays are being developed. In cold agglutinin disease (CAD), immunoglobulin M autoantibodies fix complement on the surface of red cells, resulting in extravascular hemolysis by the reticuloendothelial system. Drugs that inhibit complement activation are increasingly being used to treat these diseases. This article discusses the pathophysiology, diagnosis, and therapy for PNH, aHUS, and CAD.

Original languageEnglish (US)
Pages (from-to)385-391
Number of pages7
JournalHematology
Volume2015
Issue number1
StatePublished - Dec 5 2015

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Hemolytic Anemia
Paroxysmal Hemoglobinuria
Autoimmune Hemolytic Anemia
Hemolysis
Thrombotic Microangiopathies
Alternative Complement Pathway
Thrombotic Thrombocytopenic Purpura
Mononuclear Phagocyte System
Complement Activation
Biological Assay
Autoantibodies
Immunoglobulin M
Complement System Proteins
Mutation
Therapeutics
Pharmaceutical Preparations
Atypical Hemolytic Uremic Syndrome

ASJC Scopus subject areas

  • Medicine(all)
  • Hematology

Cite this

Complement in hemolytic anemia. / Brodsky, Robert A.

In: Hematology, Vol. 2015, No. 1, 05.12.2015, p. 385-391.

Research output: Contribution to journalArticle

Brodsky, RA 2015, 'Complement in hemolytic anemia', Hematology, vol. 2015, no. 1, pp. 385-391.
Brodsky, Robert A. / Complement in hemolytic anemia. In: Hematology. 2015 ; Vol. 2015, No. 1. pp. 385-391.
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