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Coagulation studies in thrombotic thrombocytopenic purpura, with special reference to von willebrand factor and protein S
Author(s) -
Takahashi Hoyu,
Tatewaki Wataru,
Nakamura Tadao,
Hanano Masaharu,
Wada Ken,
Shibata Akira
Publication year - 1989
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830300104
Subject(s) - plasmin , thrombotic thrombocytopenic purpura , von willebrand factor , coagulation , antithrombin , fibrinolysis , protein c , platelet , fibrinogen , protein s , chemistry , medicine , plasminogen activator , immunoelectrophoresis , endocrinology , immunology , antigen , biochemistry , heparin , enzyme
The profile of blood coagulation and fibrinolysis was studied in detail in eight patients with acute thrombotic thrombocytopenic purpura (TTP). In the majority of the patients, fibrinogen, factor XIII, antithrombin III, α 2 ‐plasmin inhibitor, plasminogen, and α 2 ‐macroglobulin were normal, whereas FDP, plasmin‐α 2 ‐plasmin inhibitor complex, and tissuetype plasminogen activator antigen were marginally or moderately elevated. Low fibronectin values were observed in four patients. Protein C and C4b‐binding protein were nearly normal, whereas total protein S and free protein S were reduced in five and six patients, respectively. A positive correlation was found between total protein S and C4 and between free protein S and C3. von Willebrand factor antigen (vWf:Ag) and ristocetin cofactor (RCof) were either normal or elevated, but RCof/vWf:Ag ratio was decreased in seven patients. Crossed immunoelectrophoresis and sodium dodecyl sulfate (SDS)‐agarose gel electrophoresis revealed that the large vWf multimers were either absent from or relatively decreased in all patients except one. In addition, one patient had unusually large vWf multimers, and a low‐molecular‐weight vWf fragment was apparently observed in three patients. These findings indicate that the intravascular generation of thrombin and plasmin was minimal in TTP and suggest that the alterations of the vWf molecule were caused not only by consumption through its participation in platelet thrombus formation but also by accelerated proteolysis. Low protein S values would be related to the immunological abnormalities underlying TTP.