Acquired haemophilia a secondary to multiple myeloma: management of a patient with a mechanical mitral valve

A 77-year-old man with a mechanical mitral valve on warfarin presented with an acute drop in haemoglobin and large spontaneous haematoma. He was found to have a new coagulopathy with initial labs notable for a prolonged activated partial thromboplastin time (APTT). Further workup revealed factor VIII levels less than 1%, abnormal mixing studies and elevated Bethesda titres, which was consistent with an acquired factor VIII inhibitor. Given his bone marrow biopsy result, which was positive for plasma cell myeloma, this coagulopathy was thought to be an acquired haemophilia A secondary to multiple myeloma. Anticoagulation was a challenge in this patient given his mechanical mitral valve and acquired haemophilia A. Although the patient was at risk of thrombosis due to a mechanical mitral valve, he had a bleeding diathesis and anaemia not responsive to transfusion. The decision was made to hold anticoagulation and the patient was started on myeloma treatment which included CyBorD, rituximab and daratumumab. After initiation of treatment APTT and factor VIII normalised. He eventually restarted anticoagulation under direction of his primary care doctor.

Giant Intramural Hematoma of the Colon in Acquired Factor VIII Inhibitor

Intramural hematoma of the colon is very rare, particularly when associated with the development of autoantibodies against factor VIII.We report a case of a 66-year-old man with abdominal pain, hematochezia and clots in the left colon, without any radiologic signs of active bleeding or bowel occlusion or analytical changes in routine coagulation screening, but with positive autoantibodies against factor VIII. The clinical instability prompted surgical exploration. An intramural hematoma of the left colon was found, and a left colectomy was performed. The patient was treated with hemoderivatives and corticosteroids with clinical improvement. The diagnosis of spontaneous intramural hematoma might be a challenge, particularly in the absence of clinical suspicion. An early recognition is essential for a positive outcome. This case highlights a rare cause of bleeding and intestinal obstruction, but also the difficulty and relevance of establishing a clinical diagnosis when diagnostic tests are not completely informative.

Acquired factor VIII inhibitor associated with chronic untreated hepatitis C

Acquired inhibitors of coagulation are antibodies that either inhibit the activity or increase the clearance of a clotting factor. A hemorrhagic diathesis is a common clinical manifestation in affected patients. Acquired factor VIII inhibitor – or acquired hemophilia A – is a rare disorder and presents similarly to hemophilia A, though patients are less likely to develop hemarthroses. This inhibition is most commonly due to autoantibodies against coagulation factor VIII. These autoantibodies often occur in pregnancy, autoimmune disorders, solid tumors, and lymphoproliferative syndromes. Several drugs, including penicillins, phenytoin, and sulfa drugs, have also been associated with antibodies to factor VIII. Chronic infection with the hepatitis C virus (HCV), in addition to various degrees of liver inflammation and fibrosis, can have extrahepatic manifestations, especially autoimmune disorders. The most common hematological complications of HCV infection are thrombocytopenia, cryoglobulinemia, and anti-cardiolipin antibodies. A few cases of factor VIII inhibitors occurring in HCV patients have been reported, with a higher incidence after prolonged treatment with interferon-α. Here, we present a case of a patient with chronic untreated HCV infection developing acquired factor VIII deficiency.