Acquired haemophilia A associated with autoimmune thyroiditis: a case report

Journal of Medical Case Reports

MG Dhammika Somarathne¹  Hannah Charlotte Copley²  Hiranya Abeysinghe¹  Nirmali Gunawardena¹  Upul GP Pathirana¹ 

[1] General Medical Unit, Teaching Hospital, Kandy, Sri Lanka;Department of Surgery, Addenbrooke’s Hospital, University of Cambridge, Cambridge, UK

关键词: Immunosuppression;    Haemophilia A;    Factor VIII inhibitors;    Bypassing agents;    Autoimmune thyroiditis;    Acquired haemophilia;   

Others  :  1180830
DOI  :  10.1186/1752-1947-8-469

 received in 2014-08-17, accepted in 2014-11-25,  发布年份 2014

【 摘 要 】

Introduction

Acquired haemophilia A is a rare life- and limb-threatening bleeding disorder if left untreated. Autoimmune thyroiditis is an autoimmune disorder that can be rarely associated with acquired haemophilia. Here we report a case of a 60-year-old woman presenting with cutaneous and muscle haematomas secondary to acquired haemophilia A in association with autoimmune thyroiditis, who was successfully treated with recombinant activated factor VII and immunosuppression.

Case presentation

A 60-year-old Sri Lankan woman with a background of longstanding hypothyroidism, diabetes mellitus, hypertension, hyperlipidaemia and bronchial asthma developed spontaneous cutaneous purpura and a limb-threatening intramuscular haematoma. Initial coagulation screening revealed prolonged activated partial thromboplastin time of 66.4 seconds (normal range 26 to -36 seconds) and time-dependent inhibitors against factor VIII. She had positive antinuclear antibody and antithyroid peroxidase (microsomal) antibody titre of over 1/80 and 1000IU/mL respectively. The diagnosis was therefore made of acquired haemophilia A in association with autoimmune thyroiditis. Acute limb-threatening bleeding was managed with recombinant activated factor VII (NovoSeven^®). Immunosuppressive treatment consisting of oral prednisone 60mg/day and cyclophosphamide 100mg/day was administered in order to remove the factor VIII inhibitor. This treatment led to normalisation of her haemostatic parameters. This case illustrates a very rare association of acquired haemophilia and autoimmune thyroiditis as well as the importance of considering acquired haemophilia as a differential diagnosis of spontaneous bleeding.

Conclusions

Acquired haemophilia should be considered in the differential diagnosis of unexplained bleeding in adults. Treatment of the acute coagulopathy with recombinant activated factor VII and immunosuppressive therapy was successful in this case.

【 授权许可】

   
2014 Pathirana et al.; licensee BioMed Central.

【 参考文献 】

• [1]Collins PW, Percy CL: Advances in the understanding of acquired haemophilia A: implications for clinical practice. Br J Haematol 2009, 148:183-194.
• [2]Collins P, Macartney N, Davies R, Lees S, Giddings J, Majer R: A population based, unselected, consecutive cohort of patients with acquired haemophilia A. Br J Haematol 2004, 124:86-90.
• [3]Franchini M, Lippi G: Acquired factor VIII inhibitors. Blood 2008, 112:250-255.
• [4]Matsumoto T, Shima M, Fukuda K, Nogami K, Giddings JC, Murakami T, Tanaka I, Yoshioka A: Immunological characterization of factor VIII autoantibodies in patients with acquired hemophilia A in the presence or absence of underlying disease. Thromb Res 2001, 104:381-388.
• [5]Huth-Kuhne A, Baudo F, Collins P: International recommendations on the diagnosis and treatment of patients with aacquired hemophilia A. Haematologica 2009, 94:566-575.
• [6]Galli M, Luciani D, Bertolini G, Barbui T: Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome. A systematic review of the literature. Blood 2003, 101:1827.
• [7]Franchini M, Gandini G, Di Paolantonio T, Mariani G: Acquired hemophilia A: a concise review. Am J Hematol 2005, 80:55.
• [8]Sumner MJ, Geldziler BD, Pedersen M, Seremetis S: Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal. Haemophilia 2007, 13:451-461.