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Thrombotic thrombocytopenic purpura and autoimmunity: a tale of shadows and suspects

Articolo
Data di Pubblicazione:
1999
Abstract:
The key pathogenic feature of TTP is the formation of platelet aggregates within the microcirculation; however, the etiology of such aggregates has been elusive for years. A large amount of evidence points to an abnormal interaction between damaged vascular endothelium and platelets, although the cause of the primary microvascular endothelial cell injury is seldom clear. The autoimmune hypothesis often recurs, and this is based on a number of observations: the claimed superiority of plasma-exchange over plasma infusion, the anecdotal report of the presence of immunocomplexes and autoantibodies in TTP patients, the efficacy of the administration of corticosteroids and other immunosuppressant agents, and the concomitant occurrence of TTP in association with autoimmune diseases, especially systemic lupus erythematosus (SLE). This review will focus on the complex relationships between TTP and humoral autoimmunity; in particular, similarities and differences between TTP, SLE and antiphospholipid (aPL) antibodies syndrome, as well as the putative role of several other antibodies directed towards endothelial cells and/or platelets, including the recently discovered anti-CD36 antibodies and antivWF-cleaving metalloprotease, will be discussed.
Tipologia CRIS:
1.1 Articolo in rivista
Keywords:
ADAM Proteins; ADAMTS13 Protein; Adult; Antibodies, Antineutrophil Cytoplasmic; Antibody Specificity; Antigens, Human Platelet; Antiphospholipid Syndrome; Autoantibodies; Autoantigens; Autoimmune Diseases; CD36 Antigens; Endothelium, Vascular; Factor VIII; Female; Humans; Immunosuppression; Lupus Erythematosus, Systemic; Male; Metalloendopeptidases; Models, Immunological; Plasma Exchange; Platelet Aggregation; Purpura, Thrombotic Thrombocytopenic; von Willebrand Factor; Autoimmunity
Elenco autori:
Porta, C; Caporali, R; Montecucco, C
Autori di Ateneo:
CAPORALI ROBERTO
MONTECUCCO CARLOMAURIZIO
PORTA CAMILLO
Link alla scheda completa:
https://iris.unipv.it/handle/11571/1283686
Pubblicato in:
HAEMATOLOGICA
Journal
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