Von Willebrand factor–cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura

• Valentina Bianchi

  From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.

• Rodolfo Robles

  From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.

• Lorenzo Alberio

  From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.

• Miha Furlan

  From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.

• Bernhard Lämmle

  From the Central Hematology Laboratory, University Hospital, Inselspital, Bern, Switzerland.

抄録

A severe deficiency in von Willebrand factor–cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a diagnosis of hemolytic uremic syndrome. However, ADAMTS13 deficiency has been claimed not to be specific for TTP, since it was observed in various thrombocytopenic and other conditions. We studied 68 patients with thrombocytopenia due to severe sepsis or septic shock (n = 17), heparin-induced thrombocytopenia (n = 16), idiopathic thrombocytopenic purpura (n = 10), or other hematologic (n = 15) or miscellaneous conditions (n = 10). Twelve of the 68 patients had subnormal levels of ADAMTS13 activity (≤ 30%), but none had less than 10%. Thus, the study showed that ADAMTS13 activity is decreased in a substantial proportion of patients with thrombocytopenia of various causes. A severe deficiency of ADAMTS13 (< 5%), identified in more than 120 patients during 1996 to 2001 in our laboratory, is specific for a thrombotic microangiopathy commonly labeled TTP.

収録刊行物

• Blood

  Blood 100 (2), 710-713, 2002-07-15

  American Society of Hematology

キーワード

• Cell Biology
• Hematology
• Immunology
• Biochemistry

詳細情報

• CRID: 1364233270623655808
• NII論文ID: 30022494113
• DOI: 10.1182/blood-2002-02-0344
• ISSN: 15280020
• ISSN: 00064971
• Web Site: http://ashpublications.org/blood/article-pdf/100/2/710/1683786/h81402000710.pdf
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