Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.

Abstract

We present the case of a woman (age: 64 years) with acute thrombotic microangiopathy due to severe acquired ADAMTS-13 (von Willebrand factor-cleaving protease) deficiency. She was successfully treated with plasma exchange therapy and glucocorticosteroids. She relapsed seven months later, and splenectomy led to lasting remission. Pathomechanisms of thrombotic thrombocytopenic purpura, especially the role of ADAMTS-13, are discussed and therapeutic measures outlined.

• Authors: A Winkler, J A Kremer Hovinga, V Bianchi, J-D Studt, B Lämmle
• Journal: Hamostaseologie (Hamostaseologie) Vol. 23 Issue 3 Pg. 103-8 (Aug 2003) ISSN: 0720-9355 [Print] Germany
• PMID: 12923577 (Publication Type: Case Reports, Journal Article)

Chemical References

• ADAM Proteins
• Metalloendopeptidases
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAM Proteins
• ADAMTS13 Protein
• Anemia, Hemolytic (blood, etiology, therapy)
• Female
• Humans
• Kidney Diseases (blood, etiology, therapy)
• Metalloendopeptidases (deficiency)
• Microcirculation (pathology)
• Middle Aged
• Plasma Exchange
• Purpura, Thrombotic Thrombocytopenic (blood, etiology, therapy)
• Thrombosis (blood, etiology, therapy)