Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von Willebrand factor-cleaving protease.

Détails

ID Serval
serval:BIB_87B1AFB386E8
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von Willebrand factor-cleaving protease.
Périodique
British Journal of Haematology
Auteur(s)
Fontana S., Gerritsen H.E., Kremer Hovinga J., Furlan M., Lämmle B.
ISSN
0007-1048 (Print)
ISSN-L
0007-1048
Statut éditorial
Publié
Date de publication
2001
Peer-reviewed
Oui
Volume
113
Numéro
1
Pages
100-102
Langue
anglais
Notes
Publication types: Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't Publication Status: ppublish
Résumé
Complete deficiency of von Willebrand factor-cleaving protease (VWF-cp) has recently been identified as a pathogenetically important factor for thrombotic thrombocytopenic purpura (TTP). Microangiopathic haemolytic anaemia (MAHA) with thrombocytopenia in patients with metastasizing neoplasms is clinically similar to TTP, however, the pathogenesis of the condition is unclear. Partial deficiency of VWF-cp in metastasizing malignancy has recently been reported in patients without MAHA. Our study shows normal or subnormal VWF-cp activity in four patients with metastasizing neoplasia-associated MAHA but, in contrast to classical TTP, no complete deficiency of VWF-cp despite the full clinical picture of MAHA.
Mots-clé
ADAM Proteins, Aged, Anemia, Hemolytic/blood, Autoantigens/blood, Bone Neoplasms/blood, Bone Neoplasms/secondary, Breast Neoplasms/blood, Female, Humans, Immunoblotting, Liver Neoplasms/blood, Liver Neoplasms/secondary, Lung Neoplasms/blood, Lung Neoplasms/secondary, Male, Metalloendopeptidases/blood, Middle Aged, Prostatic Neoplasms/blood, von Willebrand Factor/analysis, von Willebrand Factor/immunology
Pubmed
Web of science
Création de la notice
09/11/2014 17:32
Dernière modification de la notice
20/08/2019 14:46
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