[en] ADAMTS13, an important enzyme in the regulation of von Willebrand factor, may be deficient, leading to a thrombotic thrombocytopenic purpura, a subtype of thrombotic microangiopathy causing thrombocytopenia, organ failure and haemolytic anaemia. This condition may also be associated with antiphospholipid syndrome and/or systemic lupus erythematosus. We report a case of ischaemic neurological lesions due to an inaugural systemic lupus erythematosus and antiphospholipid syndrome, with the demonstration of a decreased ADAMTS13 activity without biological signs of thrombotic microangiopathy, associated with a possible COVID-19 trigger.
Disciplines :
Hematology Neurology
Author, co-author :
Dassy, Laura ✱; Centre Hospitalier Universitaire de Liège - CHU > > Service d'hématologie clinique
Aktan, David ✱; Université de Liège - ULiège > GIGA ; Centre Hospitalier Universitaire de Liège - CHU > > Service de neurologie
JASPERS, Aurélie ; Centre Hospitalier Universitaire de Liège - CHU > > Service d'hématologie clinique
SHALCHIAN, Sarvnaz ; Centre Hospitalier Universitaire de Liège - CHU > > Service de neurologie (CHR)
DELVAUX, Valérie ; Centre Hospitalier Universitaire de Liège - CHU > > Service de neurologie (CHR)
Maraite, Florence; CHR Citadelle - Centre Hospitalier Régional de la Citadelle > Ophtalmologie
Pitance, François; CHR Citadelle - Centre Hospitalier Régional de la Citadelle > Soins Intensifs
Minon Jean-Marc; CHR Citadelle - Centre Hospitalier Régional de la Citadelle > Biologie clinique
Kaye, Olivier; CHR Citadelle - Centre Hospitalier Régional de la Citadelle > Rhumatologie
✱ These authors have contributed equally to this work.
Language :
English
Title :
Severe ADAMTS13 deficit with a thrombotic thrombocytopenic purpura in a case of inaugural systemic lupus erythematosus with antiphospholipid syndrome
Alternative titles :
[fr] Déficit sévère en ADAMTS13 avec purpura thrombotique thrombocytopénique dans un cas de lupus érythémateux systémique inaugural avec syndrome des antiphospholipides
