[Multi-facetted clinical presentation of thrombotic thrombocytopenic purpura].
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathic disease. TTP is due to reduced activity of the von Willebrand factor which cleaves ADAMTS13. The disease is characterized by thrombocytopenia (<20 billion/l) intravascular Coombs-negative haemolysis and schistocytes in blood smears. Determination of the ADAMTS13-activity is now becoming available as a routine analysis. We present two cases that illustrate the multi-facetted clinical presentation under which TTP occurs. The importance of access to ADAMTS13 measurements is stressed.
Niemann
Carsten Utoft
CU
Medicinsk Haematologisk Afdeling L, Rigshospitalet, DK-2100 København Ø. niemann@dadlnet.dk.
Jurlander
Jesper
J
Daugaard
Gedske
G
Friis-Hansen
Lennart
L
D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage; D058846:Antibodies, Monoclonal, Murine-Derived; D003907:Dexamethasone / Q000008:administration & dosage; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D010956:Plasmapheresis; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D000069283:Rituximab
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D000069283:Rituximab; D003907:Dexamethasone; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human