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- Ove Juul Nielsen and Lennart Friis-Hansen.
- Klinisk Biokemisk Afdeling KB3011, Rigshospitalet, DK-2100 København Ø, Denmark.
- Ugeskr. Laeg. 2009 Nov 30;171(49):3614-20.
AbstractThe thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious sequelae.
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