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Clinical Chemistry and Laboratory Medicine (CCLM)

Published in Association with the European Federation of Clinical Chemistry and Laboratory Medicine (EFLM)

Editor-in-Chief: Plebani, Mario

Ed. by Gillery, Philippe / Greaves, Ronda / Lackner, Karl J. / Lippi, Giuseppe / Melichar, Bohuslav / Payne, Deborah A. / Schlattmann, Peter


IMPACT FACTOR 2018: 3.638

CiteScore 2018: 2.44

SCImago Journal Rank (SJR) 2018: 1.191
Source Normalized Impact per Paper (SNIP) 2018: 1.205

Online
ISSN
1437-4331
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Volume 45, Issue 1

Issues

Diagnostic approach to inherited bleeding disorders

Giuseppe Lippi
  • Sezione di Chimica e Microscopia Clinica, Dipartimento di Scienze Morfologico-Biomediche, Università degli Studi di Verona, Verona, Italy and Comitato Italiano per la Standardizzazione dei Metodi Ematologici e di Laboratorio (CISMEL), Italy
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
/ Massimo Franchini / Gian Cesare Guidi
  • Sezione di Chimica e Microscopia Clinica, Dipartimento di Scienze Morfologico-Biomediche, Università degli Studi di Verona, Verona, Italy and Comitato Italiano per la Standardizzazione dei Metodi Ematologici e di Laboratorio (CISMEL), Italy
  • Other articles by this author:
  • De Gruyter OnlineGoogle Scholar
Published Online: 2007-01-24 | DOI: https://doi.org/10.1515/CCLM.2007.006

Abstract

The appropriate development of hemostasis encompasses a delicate equilibrium between anti- and prothrombotic forces developing during three distinct phases (primary hemostasis, coagulation and fibrinolysis) that are closely linked to each other and precisely regulated to close vessel wounds, promote vascular healing and maintain vessel patency. Imbalance in each of these systems produces either hemorrhagic or thrombotic disorders. Inherited bleeding disorders, caused by quantitative or qualitative alterations of either platelets or plasma proteins involved in blood coagulation and fibrinolysis, may lead to serious and lifelong bleeding conditions, the severity of which is inversely associated with the degree of the underlying defect. Rapid and reliable identification of these pathologies is worthy of focus to allow the adoption of appropriate substitutive or supportive antihemorrhagic therapies. Evaluation of the hemorrhage-prone patient requires careful recording of the medical history, attention to pertinent physical findings and the discretionary use of laboratory resources. Owing to the low diagnostic efficiency of clinical history and examination, an appropriate and reliable laboratory approach, encompassing first- and second-line testing, is essential to screen, diagnose and monitor patients with bleeding diatheses. As both the analytical sensitivity and responsiveness of traditional coagulation assays to different abnormalities differ widely, each laboratory should establish individual guidelines based on field experience and on reagent and instrument characteristics. Emerging evidence indicates that the implementation of global coagulation tests, such as the thrombin generation assay and clot waveform analysis, would provide additional information for clinical decision-making for patients with inherited bleeding disorders.

Clin Chem Lab Med 2007;45:2–12.

Keywords: coagulation testing; hemophilia; inherited bleeding disorders; laboratory testing; platelets

References

About the article

Corresponding author: Prof. Giuseppe Lippi, Istituto di Chimica e Microscopia Clinica, Dipartimento di Scienze Morfologico-Biomediche, Ospedale Policlinico G.B. Rossi, Piazzale Scuro, 10, 37121 Verona, Italy Fax: +39-045-8201889,


Received: May 31, 2006

Accepted: September 14, 2006

Published Online: 2007-01-24

Published in Print: 2007-01-01


Citation Information: Clinical Chemical Laboratory Medicine, Volume 45, Issue 1, Pages 2–12, ISSN (Online) 1437-4331, ISSN (Print) 1434-6621, DOI: https://doi.org/10.1515/CCLM.2007.006.

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