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

Abstract

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.