Zusammenfassung
Das von-Willebrand-Syndrom ist durch quantitative, qualitative oder kombinierte
Defekte des von-Willebrand-Faktors charakterisiert und äußert sich klinisch als
hämorrhagische Diathese. Neben dem angeborenen von-Willebrand-Syndrom kommt in
seltenen Fällen bei hämatologischen Systemerkrankungen, Tumorerkrankungen, endokrinen
Störungen, kardialen Vitien oder unter medikamentöser Therapie auch eine erworbene
Form dieser Hämostasestörung vor. Pathogenetisch sind insbesondere Inhibitoren
gegen den von-Willebrand-Faktor, Adsorption an maligne Zellen, gesteigerter proteolytischer
Abbau und verminderte Synthese von Bedeutung. Die Diagnose dieses erworbenen von-Willebrand-Syndroms
wird in Zusammenschau von Anamnese, klinischer Symptomatik und Laborbefunden gestellt.
Charakteristisch ist eine erworbene hämorrhagische Diathese mit bevorzugtem
Auftreten von Schleimhautblutungen in Kombination mit verlängerter Blutungszeit
und verminderter Aktivität des von-Willebrand-Faktors. Häufig kann eine Reduktion
der hochmolekularen Multimere des von-Willebrand-Faktors nachgewiesen werden.
Grundsätzlich steht die Behandlung der Grunderkrankung im Vordergrund. Weitere
Therapieoptionen beim erworbenen von-Willebrand-Syndrom beinhalten die Applikation
des Vasopressin-Analogons Desmopressin, die Hämotherapie mit von-Willebrand-Faktor-haltigen
Faktorenkonzentraten, die intravenöse Gabe von Immunglobulinen, immunsuppressive
Maßnahmen und im Einzelfall die Immunadsorption. Die vorliegende Arbeit stellt
den aktuellen Kenntnisstand über diese seltene erworbene Hämostasestörung dar.
Insbesondere wird eine rationale Differenzialtherapie der Erkrankung diskutiert
und anhand der Grunderkrankung und assoziierten Pathomechanismen begründet.
Summary
Von Willebrand disease is characterized by an increased risk of bleeding caused
by quantitative, qualitative, or combined defects of von Willebrand factor. While
hereditary von Willebrand disease is a frequent disorder of the hemostatic system,
acquired von Willebrand syndrome is rare. This hemostatic defect is preferentially
found in patients suffering from hematological malignancies, cancer, endocrine
disorders, congenital or acquired heart valve defects, or in patients receiving
drug therapy. The pathogenesis is heterogeneous, including inhibitors against
von Willebrand factor, adsorption of von Willebrand factor onto malignant cells,
increased proteolysis, and diminished synthesis of von Willebrand factor. The
diagnostic work-up comprises the patient’s history, clinical symptoms, and laboratory
findings. An acquired bleeding tendency with preferential mucocutaneous bleeding
in combination with a prolonged bleeding time and decreased activity of von
Willebrand factor are characteristic. Further analysis often reveals a reduction
or loss of the high-molecular weight von Willebrand factor multimers. With regard
to acquired von Willebrand syndrome, therapy of the underlying disease is mandatory.
Further treatment options include application of desmopressin, hemotherapy with
von Willebrand factor containing factor concentrates, intravenous application
of immunoglobulins, immunosuppression, or immunoadsorption. This review summarizes
the current knowledge of acquired von Willebrand syndrome. In particular, we discuss
the differential therapy of this rare acquired hemostatic disorder based on the
underlying disease and associated pathomechanisms.
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Prof. Dr. Rüdiger E. Scharf
Institut für Hämostaseologie und Transfusionsmedizin, Heinrich-Heine-Universität
Düsseldorf
Moorenstraße 5
D-40225 Düsseldorf
Telefon: 0211/8117344
Fax: 0211/8116221
eMail: rscharf@uni-duesseldorf.de
