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Role of complement in the pathogenesis of thrombotic microangiopathies

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Summary

Thrombotic microangiopathies (TMAs) are rare but life-threatening disorders characterized by microvascular hemolytic anemia and acute thrombocytopenia with or without organ damage. The term TMA covers various subgroups of diseases, the pathogenesis of which is briefly summarized in this review. As highlighted here, complement activation may represent an important amalgamating process in all of these conditions, since it is able to link together activation and damage of multiple involved cell types, such as endothelial cells, platelets, and neutrophils.

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Abbreviations

ADAMTS13:

A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13

ADAMTS13 :

The gene encoding a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13

aHUS:

Atypical hemolytic uremic syndrome

AP:

Alternative pathway

C3 :

The gene encoding the complement component C3

CD46 :

The gene encoding the membrane cofactor protein

CFB :

The gene encoding complement factor B

CFH :

The gene encoding complement factor H

CFHR1 :

The gene encoding the complement factor H related protein-1

CFHR5 :

The gene encoding the complement factor H related protein-5

CFI :

The gene encoding complement factor I

DAF:

Decay-accelerating factor

DGKE:

Diacylglycerol kinase epsilon

DGKE :

The gene encoding diacylglycerol kinase epsilon

E. coli :

Escherichia coli

ET-1:

Endothelin-1

F1+2:

Plasma prothrombin fragment 1+2

Gb3:

Globotriaosylceramide

HSCT:

Hematopoietic stem cell transplantation

HUS:

Hemolytic uremic syndrome

L-FABP-1:

Liver fatty acid binding protein-1

LPS:

Lipopolysaccharide

MCP:

Membrane cofactor protein

NET:

Neutrophil extracellular trap

sC5b-9:

Soluble C5b-9 complex, or membrane attack complex

STEC-HUS:

Hemolytic uremic syndrome in connection to Shiga toxin-producing Escherichia coli infection

sTNFR1:

Soluble tumor necrosis factor receptor-1

Strep. :

Streptococcus pneumoniae

Stx:

Shiga toxin

sVCAM1:

Soluble vascular cell adhesion molecule‑1

T-antigen:

Thomsen-Friedenreich antigen

THBD :

The gene encoding thrombomodulin

TIMP‑1:

Tissue inhibitor of metalloproteinases‑1

TMA:

Thrombotic microangiopathy

TTP:

Thrombotic thrombocytopenic purpura

ULVWF:

Ultra-large form of von Willebrand factor

WPB:

Weibel-Palade body

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Authors and Affiliations

  1. George Füst Complement Diagnostic and Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary

    Eszter Trojnár, Ágnes Szilágyi, Bálint Mikes, Dorottya Csuka, György Sinkovits &  Zoltán Prohászka

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  1. Eszter Trojnár
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  2. Ágnes Szilágyi
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  3. Bálint Mikes
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  4. Dorottya Csuka
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  5. György Sinkovits
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  6. Zoltán Prohászka
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Corresponding author

Correspondence to Zoltán Prohászka.

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Conflict of interest

E. Trojnár, Á. Szilágyi, B. Mikes, D. Csuka, G. Sinkovits, and Z. Prohászka declare that they have no competing interests.

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Trojnár, E., Szilágyi, Á., Mikes, B. et al. Role of complement in the pathogenesis of thrombotic microangiopathies. memo 11, 227–234 (2018). https://doi.org/10.1007/s12254-017-0380-y

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  • DOI: https://doi.org/10.1007/s12254-017-0380-y

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