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

A Winkler; J A Kremer Hovinga; V Bianchi; J-D Studt; B Lämmle

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

Hamostaseologie. 2003 Aug;23(3):103-8.

Authors

A Winkler¹, J A Kremer Hovinga, V Bianchi, J-D Studt, B Lämmle

Affiliation

¹ Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland.

PMID: 12923577

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.

Publication types

Case Reports

MeSH terms

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

Substances

ADAM Proteins
Metalloendopeptidases
ADAMTS13 Protein
ADAMTS13 protein, human