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Plasminogen activator inhibitor (PAI‐1) antigen levels in primary TTP and secondary TTP post‐bone marrow transplantation
Author(s) -
Anthony Maya T.,
Zeigler Zella R.,
Lister John,
Raymond Jane M.,
Shadduck Richard K.,
Kramer Robert E.,
Gryn Jeffrey F.,
Rintels P. B.,
Besa Emmanuel C.,
George James N.,
Silver Bernard,
Joyce Robert,
Bodensteiner David
Publication year - 1998
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/(sici)1096-8652(199809)59:1<9::aid-ajh3>3.0.co;2-t
Subject(s) - medicine , thrombotic thrombocytopenic purpura , bone marrow , gastroenterology , quartile , transplantation , platelet , immunology , surgery , confidence interval
Our objectives were to measure and compare plasminogen activator inhibitor levels (PAI‐1) in primary adult thrombotic thrombocytopenic purpura (TTP) and in secondary TTP associated with bone marrow transplantation (BMT)‐TTP. PAI‐1 antigen levels were measured by an enzyme linked immunosorbent assay on platelet poor plasma samples obtained from patients at the time of diagnosis of the TTP disorder and from a group of normal volunteers. The samples were frozen at –70°C. Patients with TTP secondary to bone marrow transplantation had their grade determined by percentage fragmented cells and lactate dehydrogenase levels. The primary TTP samples were contributed by investigators in the multi‐institutional North American TTP Group, and the bone marrow transplant samples were obtained from an adult bone marrow transplant program. Nineteen patients with adult TTP, and 47 patients with bone marrow transplant‐TTP were evaluated. Of the latter, 14 had Grade 2, 13 had Grade 3, and 20 had Grade 4 BMT‐TTP. PAI‐1 levels were elevated compared to control volunteers in both primary adult TTP and BMT‐TTP, P < 0.001. Levels did not differ from normal in Grade 2 BMT‐TTP (median = 16 ng/ml; quartiles = 9–20). PAI‐1 levels were similar in primary TTP (median = 32 ng/ml; quartiles = 25–51) and Grade 3 BMT‐TTP (median = 35 ng/ml; quartiles = 19–48 ng/ml), P = 0.7. However, PAI‐1 levels were significantly higher in Grade 4 BMT‐TTP (median = 83 ng/ml; quartiles = 60–143) than Grade 3 BMT‐TTP, and primary TTP, P < 0.001. PAI‐1 levels are high in primary TTP and secondary bone marrow transplant‐TTP (Grades 3–4). In contrast, normal levels are seen in Grade 2 BMT‐TTP, which is a self‐limited disorder. Therefore, high PAI‐1 levels may contribute to hypofibrinolysis in the pathogenesis of primary TTP and of moderate to severe TTP (Grades 3–4) following bone marrow transplantation. Am. J. Hematol. 59:9–14, 1998. © 1998 Wiley‐Liss, Inc.