Schistocytic anaemia, severe thrombocytopenia, and renal dysfunction: thrombotic microangiopathy due to severe acquired ADAMTS-13 deficiency. Case 2.
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.
Winkler
A
A
Central Haematology Laboratory, Inselspital, University Hospital, Bern, Switzerland.
Kremer Hovinga
J A
JA
Bianchi
V
V
Studt
J-D
JD
Lämmle
B
B
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000743:Anemia, Hemolytic / Q000097:blood / Q000209:etiology* / Q000628:therapy; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000097:blood / Q000209:etiology / Q000628:therapy; D008666:Metalloendopeptidases / Q000172:deficiency*; D008833:Microcirculation / Q000473:pathology; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000209:etiology* / Q000628:therapy; D013927:Thrombosis / Q000097:blood / Q000209:etiology / Q000628:therapy
D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human