Idiopathic Acquired Factor VIII deficiency presenting with compartment syndrome: A case report and Literature Review

Idiopathic Acquired Factor VIII deficiency presenting with compartment syndrome: A case report and Literature Review

Khalid E. Ahmed1, Amna Gameil2, Marcus D. Lance3, Arshad H. Chanda3, Lubna Riaz2, Anas Hamad4, Ahmed A. Shible4, Shehab F. Mohamed2 

1Department of Medicine Hamad Medical Corporation; 2Medical oncology, Hematology section, NCCCR, Hamad Medical Corporation; 3Department of Anaesthesiology, surgical intensive care and perioperative medicine, Hamad medical corporation; 4Pharmacy department, NCCCR, Hamad medical corporation

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Haemophilia is a disorder that affects the ability of the blood to form clots. The congenital form of the disease is the most prevalent, is inherited as X-linked recessive and it causes deficiency of clotting Factor VIII or IX. clinically it presents with joint bleeding. Its counterpart, acquired haemophilia is a rare condition that usually presents with cutaneous, soft tissue or internal bleeding.
The pathophysiology of the disease is centred on the formation of auto antibodies which inactivate factor VIII. Haematologically this is reflected as a prolonged aPTT with normal PT and failure of mixing studies to correct aPTT to more than 50%. To confirm the diagnosis Bethesda assay has to be performed to detect the presence of factor inhibitors. In half of the cases it is associated with an underlying condition such as autoimmune diseases, malignancy, pregnancy or infections.
The mainstay treatment is to control the bleeding with bypassing agents such as recombinant factor VIIa or Factor VIII inhibitor bypassing agent as well as eradicating the inhibitor with immunosuppressive and/or cytotoxic agents. Here we report a patient with idiopathic acquired haemophilia who presented with a thigh compartment syndrome. He was successfully treated with fasciotomy, bypassing agents and immunosuppressive therapy.

Keywords: Acquired, Haemophilia, Factor VIII, compartment syndrome

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How to cite this article:

Khalid E. Ahmed, Amna Gameil, Marcus D. Lance, Arshad H. Chanda, Lubna Riaz, Anas Hamad, Ahmed A. Shible, Shehab F. Mohamed. Idiopathic Acquired Factor VIII deficiency presenting with compartment syndrome: A case report and Literature Review . International Journal of Case Reports, 2020 4:115. DOI: 10.28933/ijcr-2020-01-2405


1. Kessler CM, Knobl P: Acquired haemophilia: An overview for clinical practice. Eur J Haematol, 2015; 95(Suppl. 81): 36–44
2. Flisinki M, Windyga J, Stefanska E et al: Acquired haemophilia: A case report. Pol Arch Med Wewn, 2008; 118(4): 228–33
3. 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(5): 1870–77
4. Collins P, Baudo F, Huth-Kuhne A et al: Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res Notes, 2010; 3: 161
5. Lee CP, Khalid BB: A case series of acquired haemophilia in a Malaysian hospital: unpredictably rare medical emergency. Oxf Med Case Reports, 2015; 2015(10): 330–32
6. 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(4): 622–31
7. Ma AD, Carrizosa D: Acquired factor VIII Inhibitors: Pathophysiology and treatment. Hematology Am Soc Hematol Educ Program, 2006: 432–37
8. Franchini M, Vaglio S, Marano G et al: Acquired hemophilia A: A review of recent data and new therapeutic options. Hematology, 2017; 22(9): 514–20
9. Sakurai Y, Takeda T: Acquired hemophilia A: A frequently overlooked autoimmune hemorrhagic disorder. J Immunol Res, 2014; 2014: 32067

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