Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.

Détails

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Etat: Serval
Version: Final published version
ID Serval
serval:BIB_F0C8194CAA01
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory.
Périodique
Swiss medical weekly
Auteur(s)
Studt J.D., Kremer Hovinga J.A., Alberio L., Bianchi V., Lämmle B.
ISSN
1424-7860 (Print)
ISSN-L
0036-7672
Statut éditorial
Publié
Date de publication
14/06/2003
Peer-reviewed
Oui
Volume
133
Numéro
23-24
Pages
325-332
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Résumé
Severe deficiency of von Willebrand factor-cleaving protease (ADAMTS-13) activity (<5% of normal) is specific for classical thrombotic thrombocytopenic purpura (TTP), a disorder presenting with thrombocytopenia, microangiopathic haemolytic anaemia and often with organ dysfunction such as neurological symptoms, renal failure, and fever. A certain, though according to several case series, variable percentage of patients with clinically diagnosed TTP and most patients with other forms of thrombotic icroangiopathies (TMA) do not show severe ADAMTS-13 deficiency.
We determined ADAMTS-13 activity in 508 plasma samples of 309 patients referred to our laboratory in 2001 and 2002. Plasma samples with ADAMTS-13 activity <5% were additionally tested for the presence of inhibitory antibodies. Patients were assigned to ten predefined clinical categories according to information provided in the referral letter (TMA not specified; TMA associated with neoplasia or chemotherapy; TMA following haematopoietic stem cell transplantation; TMA with additional disorder; idiopathic TTP; haemolytic-uraemic syndrome (HUS) not specified; HUS with diarrhoea prodrome; atypical HUS; other haematological disorder; no clinical information available).
We detected 50 (16%) patients with severe ADAMTS-13 deficiency. Forty-four (88%) of these patients had been classified as idiopathic TTP, 2 as neoplasia- or chemotherapy-associated, and 4 as non-specified TMA. Among the patients labelled as acute idiopathic TTP, the prevalence of severe ADAMTS-13 deficiency was 63% (44/70). Inhibitory antibodies were found in 31 (62%) patients with ADAMTS-13 activity <5%. Of the 44 patients with acute idiopathic TTP, at initial presentation or at relapse, with ADAMTS-13 activity <5%, 11 were identified to have (probable) constitutional severe ADAMTS-13 deficiency.
Severe ADAMTS-13 deficiency is found in about 60% of patients diagnosed with idiopathic TTP but in none of 111 diagnosed with HUS. Plasma ADAMTS-13 activity <5%, however, does not identify all patients clinically diagnosed with TTP. Detection of inhibitory antibodies against ADAMTS-13 helps to differentiate between acquired and constitutional forms of TTP, which may be important for treatment strategies.

Mots-clé
ADAM Proteins, ADAMTS13 Protein, Adolescent, Adult, Aged, Aged, 80 and over, Child, Female, Humans, Laboratories, Male, Metalloendopeptidases/blood, Middle Aged, Purpura, Thrombotic Thrombocytopenic/diagnosis, Purpura, Thrombotic Thrombocytopenic/enzymology
Pubmed
Web of science
Création de la notice
10/02/2015 12:02
Dernière modification de la notice
15/06/2018 15:11
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