Qualitative platelet defect and thrombohaemorrhagic complications in a patient with polycythaemia vera

Summary

We describe a 67-year-old patient with polycythaemia vera and pathological functional platelet studies. He not only suffered a transient ischaemic attack despite taking of antiplatelet agents, but also showed bleeding diathesis with cerebral bleeding and spontaneous suffusions. Platelet function studies and clinical findings improved after phlebotomy and cytoreductive treatment with hydroxyurea.

Thrombosis and haemorrhage account predominantly for morbidity and mortality in patients with polycythaemia vera. The pathophysiological mechanisms to explain thrombosis and bleeding in patients with myeloproliferative disorders including polycythaemia vera were intensively studied. However, up to now no clear correlation of laboratory findings in relation to clinical history of thrombosis and bleeding was demonstrated. In this report the most important pathophysiological mechanisms and therapy with antiplatelet agents are discussed.

Zusammenfassung

Ein 67-jähriger Patient mit Polycythaemia rubra vera erlitt trotz Einnahme eines Thrombozytenaggregationshemmers rezidivierende zerebrale Durchblutungsstörungen. Zusätzlich erlitt er eine zerebrale Blutung und wiederholte, spontane Suffusionen als Zeichen einer hämorrhagischen Diathese. Die Thrombozytenaggregationstests zeigten eindeutig eine Plättchenfunktionsstörung. Sowohl die Labor- wie auch die klinischen Befunde verbesserten sich nach Einleitung der Behandlung mit Aderlässen und Hydroxyurea. Thromboembolien und Blutungen sind hauptsächlich verantwortlich für Morbidität und Mortalität von Patienten mit Polycythaemia rubra vera.

Die pathophysiologischen Mechanismen, die Thrombose- und Blutungsneigung verursachen sollen, wurden in zahlreichen Studien untersucht. Eine eindeutige Korrelation zwischen den beschriebenen Laborbefunden und der klinischen Symptomatik konnte noch nicht gezeigt werden. In diesem Artikel werden die wichtigsten pathophysiologischen Mechanismen, u. a. im Hinblick auf die Behandlung mit Thrombozytenaggregationshemmern, diskutiert.

Keywords

Polycythaemia vera - platelet function - thrombosis - antiplatelet agents

Schlüsselwörter

Polyzythaemia rubra vera - Plättchenfunktionsstörung - Thrombose - Thrombozytenaggregationshemmer

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