The Diagnostic Assessment of Platelet Function Defects - Part 2: Update on Platelet Disorders

Karina Althaus; Gero Hoepner; Barbara Zieger; Florian Prüller; Anna Pavlova; Doris Boeckelmann; Ingvild Birschmann; Jens Müller; Heiko Rühl; Ulrich Sachs; Beate Kehrel; Werner Streif; Peter Bugert; Carlo Zaninetti; Nina Cooper; Harald Schulze; Ralf Knöfler; Tamam Bakchoul; Kerstin Jurk

doi:10.1055/a-2404-0216

Hämostaseologie, Table of Contents

Hamostaseologie
DOI: 10.1055/a-2404-0216

Review Article

The Diagnostic Assessment of Platelet Function Defects - Part 2: Update on Platelet Disorders

Karina Althaus

¹Medical Faculty of Tübingen, Institute for Clinical and Experimental Transfusion Medicine, Tübingen, Germany

²Center for Clinical Transfusion Medicine, University Hospital of Tübingen, Tübingen, Germany

,

Gero Hoepner

²Center for Clinical Transfusion Medicine, University Hospital of Tübingen, Tübingen, Germany

³Department of Anaesthesiology and Intensive Care, University Hospital Tübingen, Tübingen, Germany

,

Barbara Zieger

⁴Department of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Freiburg, Germany

,

Florian Prüller

⁵Klinisches Institut für Medizinische und Chemische Labordiagnostik, Medizinische Universität Graz, Graz, Austria

,

Anna Pavlova

⁶Institute of Experimental Haematology and Transfusion Medicine (IHT), University Hospital Bonn, Bonn, Germany

,

Doris Boeckelmann

⁴Department of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Freiburg, Germany

,

Ingvild Birschmann

⁷Herz- und Diabeteszentrum Nordrhein-Westfalen, Universitätsklinik der Ruhr-Universität Bochum, Institut für Laboratoriums- und Transfusionsmedizin, Bochum, Germany

,

Jens Müller

⁶Institute of Experimental Haematology and Transfusion Medicine (IHT), University Hospital Bonn, Bonn, Germany

,

Heiko Rühl

⁶Institute of Experimental Haematology and Transfusion Medicine (IHT), University Hospital Bonn, Bonn, Germany

,

Ulrich Sachs

⁸Institute for Clinical Immunology and Transfusion Medicine, Justus Liebig University, Giessen, Germany

,

Beate Kehrel

⁹Department of Anaesthesiology and Intensive Care, Experimental and Clinical Haemostasis, University-Hospital Munster, Münster, Germany

,

Werner Streif

¹⁰Kinder- und Jugendheilkunde, Innsbruck Medical University, Innsbruck, Austria

,

Peter Bugert

¹¹Medical Faculty Mannheim, Institute of Transfusion Medicine and Immunology, Heidelberg University, Mannheim, Germany

,

Carlo Zaninetti

¹²Institute of Immunology and Transfusion Medicine, Universitätsmedizin Greifswald, Greifswald, Germany

,

Nina Cooper

⁸Institute for Clinical Immunology and Transfusion Medicine, Justus Liebig University, Giessen, Germany

,

Harald Schulze

¹³Institute of Experimental Biomedicine, Universitätsklinikum Würzburg, Würzburg, Germany

,

Ralf Knöfler

¹⁴Department of Paediatric Haemostaseology, Dresden University Hospital, Dresden, Germany

,

Tamam Bakchoul

¹Medical Faculty of Tübingen, Institute for Clinical and Experimental Transfusion Medicine, Tübingen, Germany

²Center for Clinical Transfusion Medicine, University Hospital of Tübingen, Tübingen, Germany

,

Kerstin Jurk

¹⁵Center for Thrombosis and Hemostasis, University Medical Center Mainz, Mainz, Germany

› Author Affiliations

Abstract

Congenital platelet disorders are rare and targeted treatment is usually not possible. Inherited platelet function disorders (iPFDs) can affect surface receptors and multiple platelet responses such as defects of platelet granules, signal transduction, and procoagulant activity. If iPFDs are also associated with a reduced platelet count (thrombocytopenia), it is not uncommon to be misdiagnosed as immune thrombocytopenia. Because the bleeding tendency of the different platelet disorders is variable, a correct diagnosis of the platelet defect based on phenotyping, function analysis, and genotyping is essential, especially in the perioperative setting. In the case of a platelet receptor deficiency, such as Bernard–Soulier syndrome or Glanzmann thrombasthenia, not only the bleeding tendency but also the risk of isoimmunization after platelet transfusions or pregnancy has to be considered. Platelet granule disorders are commonly associated with either intrinsically quantitative or qualitative granule defects due to impaired granulopoiesis, or granule release defects, which can also affect additional signaling pathways. Functional platelet defects require expertise in the clinical bleeding tendency in terms of the disorder when using antiplatelet agents or other medications that affect platelet function. Platelet defects associated with hematological-oncological diseases require comprehensive information about the patient including the clinical implication of the genetic testing. This review focuses on genetics, clinical presentation, and laboratory platelet function analysis of iPFDs with or without reduced platelet number. As platelet defects affecting the cytoskeleton usually show thrombocytopenia, but less impaired or normal platelet functional responses, they are not specifically addressed.

Keywords

diagnosis management - inherited coagulation disorders - inherited/acquired platelet disorders - platelet pathology/inherited

Full Text

References

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Supplementary Material