Hemmkörperhämophilie: aktuelle Aspekte

Zusammenfassung

Die erworbene Hämophilie A stellt die häufigste Hemmkörperhämophilie dar. Sie kann idiopathisch, in Zusammenhang mit anderen Erkrankungen oder post partum auftreten. In einem Großteil der Fälle treten ausgeprägte Blutungszeichen auf, die lebensbedrohlich verlaufen können. Wichtige Differentialdiagnosen werden genannt. Die Diagnose wird ausgehend von der verlängerten aPTT durch Plasmamischversuche und den Bethesda-Test gestellt. Die Therapie erfolgt einerseits neben lokalen Maßnahmen zur Blutungskontrolle durch Gerinnungsfaktorpräparate sowie Tranexamsäure zur Blutstillung sowie andererseits durch Immunsuppression mit Glukokortikoiden, Rituximab und/oder Cyclophosphamid zur Elimination des inhibitorischen Autoantikörpers. Alternative Immunsuppressive Therapieoptionen für refraktäre Fälle werden aufgezeigt. Die Prognose scheint vor allem davon abhängig zu sein wie stark Faktor VIII initial reduziert ist.

Abstract

Acquired haemophilia A is the most common form of inhibitor-induced aquired haemophilia. It can occur idiopathically, in association with other diseases or post partum. In the majority of cases, there are pronounced bleeding signs that can be life-threatening. Important differential diagnoses are mentioned. The diagnosis is made on the basis of prolonged aPTT by plasma mixing tests and the Bethesda test. As treatment, local measures, coagulation factor preparations and tranexamic acid are used to stop bleeding, and immunosuppression with glucocorticoids, rituximab and cyclophosphamide to eliminate the inhibitory autoantibody. Further immunosuppressive treatment options to treat refractory cases are mentioned. Prognosis seems to depend mainly on the extent to which factor VIII is initially reduced.

Schlüsselwörter

Hemmkörperhämophilie - Diagnose - Therapie

Key words

aquired hemophilia - diagnosis - treatment

Publication History

Article published online:
18 August 2022

• Literatur

• 1 Collins P, Macartney N, Davies R. et al. A population based, unselected, consecutive cohort of patients with acquired haemophilia A. Br J Haematol 2004; 124: 86
• 2 Collins PW, Hirsch S, Baglin TP. et al. Acquired hemophilia A in the United Kingdom: a 2–year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood 2007; 109: 1870
• 3 Hauser I, Schneider B, Lechner K. Post-partum factor VIII inhibitors. A review of the literature with special reference to the value of steroid and immunosuppressive treatment. Thromb Haemost 1995; 73: 1
• 4 Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost 1981; 45: 200
• 5 Knoebl P, Marco P, Baudo F. et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost 2012; 10: 622-631
• 6 Plüß M, Mitteldorf C, Szuszies CJ. et al. Case Report: Acquired Haemophilia A Following mRNA-1273 Booster Vaccination Against SARS-CoV-2 With Concurrent Diagnosis of Pleomorphic Dermal Sarcoma. Front Immunol 2022; 13: 868133
• 7 Guerra JD, Gowarty J, Buess J. et al. A Case of Acquired Hemophilia A in a Patient with Exposure to COVID-19. Case Rep Hematol 2022; 2022: 9494249
• 8 Coppola A, Franchini M, Tripodi A. et al. Acquired haemophilia A: Italian Consensus Recommendations on diagnosis, general management and treatment of bleeding. Blood Transfus. 2022 Jan 20; Epub ahead of print.
• 9 Tiede A, Collins P, Knoebl P. et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica 2020; 105: 1791-1801
• 10 Thomas VM, Abou-Ismail MY, Lim MY. Off-label use of emicizumab in persons with acquired haemophilia A and von Willebrand disease: A scoping review of the literature. Haemophilia 2022; 28: 4-17
• 11 Hansenne A, Hermans C. Emicizumab in acquired haemophilia A: about two clinical cases and literature review. Ther Adv Hematol 2021; 12 20406207211038193
• 12 Wang LY, Shen Y, Zeng HQ. et al. Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series. Medicine (Baltimore) 2021; 100: e26587
• 13 Esteves Pereira M, Bocksrucker C, Kremer Hovinga JA. et al. Immunoadsorption for the Treatment of Acquired Hemophilia: New Observational Data, Systematic Review, and Meta-Analysis. Transfus Med Rev 2021; 35: 125-134
• 14 Wang LY, Shen Y, Zeng HQ. et al. Feasibility of therapeutic plasma exchange-based combination therapy in the treatment of acquired hemophilia A: A retrospective 6 case series. Medicine (Baltimore) 2021; 100: e26587
• 15 Tiede A, Klamroth R, Scharf RE. et al. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study. Blood 2015; 125: 1091-1097