Hämophilie A und B beim Hund^[*]

Zusammenfassung

Diese Übersichtsarbeit präsentiert anhand eigener klinischer Erfahrungen und der Literatur Aspekte von Ätiologie, Pathogenese, klinischem Bild, Diagnose und Therapie der Hämophilie A und B beim Hund. Im Vergleich zum Menschen zeigen Hunde bei einer definierten Faktor-Restaktivität schwerere Blutungssymptome (u. a. subkutane, intramuskuläre und intraartikuläre Blutungen nach inadäquatem Trauma mit der Folge von Lähmungen und Lahmheiten, exzessive Blutungen bei Zahnwechsel, Venenpunktion und Operationen). Erfreulicherweise sind nun in Deutschland für einige Rassen (Hämophilie B beim Rhodesian Ridgeback, Hämophile A beim Havaneser) kommerziell Gentests verfügbar, die die konventionelle Einzelfaktormessung ergänzen und die Erkennung von Trägerhündinnen erleichtern. Bei der Therapie von Blutungskrisen steht neben lokal blutstillenden Maßnahmen nach wie vor die Substitutionsbehandlung mit frischem Plasma oder gefrorenem Frischplasma im Vordergrund. Hingegen haben sich Erwartungen, die auch beim Menschen in die zeitnahe klinische Verfügbarkeit der Gentherapie gesetzt wurden, bislang nicht erfüllt.

Summary

Based on own clinical experiences and the literature, this review article elaborates on aspects of aetiology, pathogenesis, clinical signs, diagnosis and therapy of haemophilia A and B in dogs. When compared to humans, dogs reveal more severe haemorrhagic symptoms at a defined residual factor activity (e. g. subcutaneous, intramuscular, and intraarticular haemorrhages after inappropriate trauma resulting in lameness and paralysis, excessive haemorrhage during second dentition, venal puncture, and surgery). Fortunately, genetic tests are now commercially available in Germany for selected breeds (haemophilia B in Rhodesian Ridgebacks; haemophilia A in Havaneses), which complement the conventional individual factor activity measurements and facilitate the detection of female carrier dogs. Treatment of bleeding crises is still mainly based on substitution therapy with fresh or fresh frozen plasma in addition to local haemostatic measures. In contrast, expectations regarding the timely clinical availability of gene therapy (particularly in humans) have not yet been fulfilled.

Schlüsselwörter

Hämophilie A - Hämophilie B - Hund - Rassen - Diagnostik - Gentest - Therapie - Literaturübersicht

Key words

Haemophilia A - haemophilia B - dog - breeds - diagnosis - genetic testing - therapy - literature review

^* Herrn Prof. Dr. Ingo Nolte zum 60. Geburtstag gewidmet.

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