Literature DB >> 3893523

Platelet aggregation occurs in congenital afibrinogenaemia despite the absence of fibrinogen or its fragments in plasma and platelets, as demonstrated by immunoenzymology.

J Soria, C Soria, J Y Borg, M Mirshahi, H Piguet, P Tron, C Fessard, J P Caen.   

Abstract

Platelet aggregation of an afibrinogenaemic patient's platelet rich plasma (PRP) was greatly decreased when ADP was used for stimulation. In the presence of collagen or arachidonic acid the changes in light transmission recorded during platelet aggregation of patient's PRP were similar to those observed with normal PRP but the size of aggregates appeared to be smaller in comparison with those observed with normal platelets. In addition, thrombin-induced aggregation of washed platelets was similar to normal platelets. The interpretation was made possible because the fibrinogen level in plasma and in platelets was found to be almost nil as demonstrated by both an Elisa procedure described here and the determination of fibrinopeptide A (fpA). Furthermore, fibrinogen fragments, which could result from abnormal synthesis and therefore replace fibrinogen in platelet aggregation, were undetectable by immunological analysis using specific antibodies against A alpha, B beta and gamma chains and 10 different monoclonal antibodies against fibrin degradation products.

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Year:  1985        PMID: 3893523     DOI: 10.1111/j.1365-2141.1985.tb07447.x

Source DB:  PubMed          Journal:  Br J Haematol        ISSN: 0007-1048            Impact factor:   6.998


  5 in total

1.  Budd-Chiari syndrome in an afibrinogenemic patient: a paradoxical complication.

Authors:  Nevin Oruc; Yaman Tokat; Refik Killi; Murat Tombuloglu; Tankut Ilter
Journal:  Dig Dis Sci       Date:  2006-02       Impact factor: 3.199

2.  Fibrinogen-independent platelet adhesion and thrombus formation on subendothelium mediated by glycoprotein IIb-IIIa complex at high shear rate.

Authors:  H J Weiss; J Hawiger; Z M Ruggeri; V T Turitto; P Thiagarajan; T Hoffmann
Journal:  J Clin Invest       Date:  1989-01       Impact factor: 14.808

3.  von Willebrand factor interaction with the glycoprotein IIb/IIa complex. Its role in platelet function as demonstrated in patients with congenital afibrinogenemia.

Authors:  L De Marco; A Girolami; T S Zimmerman; Z M Ruggeri
Journal:  J Clin Invest       Date:  1986-04       Impact factor: 14.808

4.  Association of fibrinogen with human platelets pretreated with chymotrypsin or aggregated with ADP or thrombin: an immunocytochemical study.

Authors:  H Suzuki; R L Kinlough-Rathbone; M A Packham; H Yamazaki; J F Mustard
Journal:  Br J Exp Pathol       Date:  1989-08

Review 5.  Treatment of congenital fibrinogen deficiency: overview and recent findings.

Authors:  Konstantinos Tziomalos; Sofia Vakalopoulou; Vassilios Perifanis; Vassilia Garipidou
Journal:  Vasc Health Risk Manag       Date:  2009-10-12
  5 in total

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