New insights in the pathogenesis of autoimmune hemolytic anemia

Research output: Contribution to journalArticle

Abstract

Autoimmune hemolytic anemia (AIHA) is caused by the increased destruction of red blood cells (RBCs) by anti-RBC autoantibodies with or without complement activation. RBC destruction may occur both by a direct lysis through the sequential activation of the final components of the complement cascade (membrane attack complex), or by antibody-dependent cell-mediated cytotoxicity (ADCC). The pathogenic role of autoantibodies depends on their class (the most frequent are IgG and IgM), subclass, thermal amplitude (warm and cold forms),as well as affinity and efficiency in activating complement. Several cytokines and cytotoxic mechanisms (CD8+ T and natural killer cells) are further involved in RBC destruction. Moreover, activated macrophages carrying Fc receptors may recognize and phagocyte erythrocytes opsonized by autoantibodies and complement. Direct complement-mediated lysis takes place mainly in the circulations and liver, whereas ADCC, cytotoxicity, and phagocytosis occur preferentially in the spleen and lymphoid organs. The degree of intravascular hemolysis is 10-fold greater than extravascular one. Finally, the efficacy of the erythroblastic compensatory response can greatly influence the clinical picture of AIHA. The interplay and relative burden of all these pathogenic mechanisms give reason for the great clinical heterogeneity of AIHAs, from fully compensated to rapidly evolving fatal cases.

Original languageEnglish
Pages (from-to)287-293
Number of pages7
JournalTransfusion Medicine and Hemotherapy
Volume42
Issue number5
DOIs
Publication statusPublished - Dec 1 2015

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Autoimmune Hemolytic Anemia
Erythrocytes
Autoantibodies
Antibody-Dependent Cell Cytotoxicity
Liver Circulation
Complement Membrane Attack Complex
Natural Killer T-Cells
Fc Receptors
Complement Activation
Phagocytes
Hemolysis
Phagocytosis
Immunoglobulin M
Spleen
Immunoglobulin G
Hot Temperature
Macrophages
Cytokines

Keywords

  • Autoantibodies
  • Autoimmune hemolytic anemia
  • Cytokines

ASJC Scopus subject areas

  • Hematology
  • Immunology and Allergy

Cite this

New insights in the pathogenesis of autoimmune hemolytic anemia. / Barcellini, Wilma.

In: Transfusion Medicine and Hemotherapy, Vol. 42, No. 5, 01.12.2015, p. 287-293.

Research output: Contribution to journalArticle

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