Prolonged inhibition of von Willebrand factor-cleaving protease after splenectomy in a 22-year-old patient with acute and plasma refractory thrombotic thrombocytopenic purpura

British Journal of Haematology

Volumn 118, Issue 1, 2002, Pages 271-274

  Langer, Florian ^a   Bergmann, Frauke ^b   Budde, Ulrich ^b   Hegewisch Becker, Susanna ^a   Hossfeld, Dieter Kurt ^a  

^a UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

^b Arndt and Partner   (Germany)

Author keywords

Splenectomy; Thrombotic thrombocytopenic purpura; Unusually large VWF multimers; VWF cleaving protease

Indexed keywords

AZATHIOPRINE; PREDNISONE; PROTEINASE; UNCLASSIFIED DRUG; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ADULT; ARTICLE; CASE REPORT; CLINICAL FEATURE; ENZYME ACTIVITY; ENZYME DEFICIENCY; ENZYME INHIBITION; FEMALE; FOLLOW UP; HUMAN; PLASMAPHERESIS; PRIORITY JOURNAL; RELAPSE; SPLENECTOMY; THROMBOCYTE COUNT; THROMBOTIC THROMBOCYTOPENIC PURPURA; TREATMENT OUTCOME;

ADAM PROTEINS; ADULT; FEMALE; FOLLOW-UP STUDIES; HUMANS; METALLOENDOPEPTIDASES; PLATELET COUNT; PURPURA, THROMBOTIC THROMBOCYTOPENIC; SPLENECTOMY; VON WILLEBRAND FACTOR;

EID: 0036062420     PISSN: 00071048     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1365-2141.2002.03566.x     Document Type: Article

References (12)

1. Von Willebrand factor-cleaving protease activity in congenital thrombotic thrombocytopenic purpura
2. Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease
3. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family
4. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura
5. Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome
6. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura
7. Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: A tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP)
8. HLA-DR53 protects against thrombotic thrombocytopenic purpura/adult hemolytic uremic syndrome
9. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura
10. Splenectomy during partial remission in thrombotic thrombocytopenic purpura with prolonged plasma exchange dependency
11. Management of thrombotic thrombocytopenic purpura
12. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura