Acquired von Willebrand syndrome in myeloproliferative disorder

Summary

We present a woman (age: 57 years) with an excessive bleeding episode under acetylsalicylic acid after bone marrow puncture due to an acquired von Willebrand syndrome (avWS) in the context of a myeloproliferative disorder. The laboratory features showed a high platelet concentration and a qualitative defect of von Willebrand factor (vWF) with a low normal vWF ristocetin cofactor activity, a normal vWF antigen and a decrease of the larger vWF multimers in plasma.

The exact mechanism of avWS is still incompletely resolved. Myeloproliferative diseases are one of several underlying disorders that may cause avWS. The diagnosis of the underlying disease is important because its treatment may lead to an improvement of the vWF abnormality. For symptomatic treatment of bleeding, desmopressin, vWF concentrate infusion, intravenous immunoglobulin and/or fibrinolysis inhibitors can be tried.

Zusammenfassung

Der Fall einer 57-jährigen Frau mit einer Blutungsepisode nach Knochenmarkpunktion unter Azetylsalizylsäure wird vorgestellt. Die Laboruntersuchungen zeigten eine Thrombozytose und einen qualitativen Defekt des von-Willebrand-Faktors (vWF) mit einer tiefnormalen vWF-Ristocetin-Kofaktoraktivität, einem normalen vWF-Antigen und einer Verminderung der hochmolekularen vWF-Multimere, entsprechend einem akquirierten von-Willebrand-Syndrom (avWS) im Rahmen einer myeloproliferativen Krankheit.

Der exakte Mechanismus des avWS ist nicht vollständig geklärt. Myeloproliferative Erkrankungen gehören zu den möglichen Ursachen. Die Identifizierung der Grundkrankheit ist wichtig, weil deren erfolgreiche Behandlung das avWS beheben kann. Für eine symptomatische avWS-Behandlung stehen Desmopressin, vWF-haltige Faktor-VIII-Konzentrate, intravenös zu verabreichende Immunglobuline und Fibrinolysehemmer zur Verfügung.

Keywords

Myeloproliferative disorder - von Willebrand factor - acquired von Willebrand syndrome - bleeding disorder

Schlüsselwörter

Myeloproliferative Erkrankungen - von-Willebrand-Faktor - akquiriertes von-Willebrand-Syndrom - hämorrhagische Diathese - Blutungsneigung

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