No abstract

Acquired factor VIII (FVIII) inhibitor in patients without haemophilia A is an uncommon disorder, occurring at a rate of 0.2-1.0 case per million each year [1]. Data on associated diseases are not always available and, in a consecutive cohort of patients in the UK, acquired haemophilia may be idiopathic in approximately 60% of the patients [2].A variety of clinical conditions, such as drug hypersensitivity, solid tumours, lymphoproliferative diseases, postpartum status, skin disorders, autoimmune disorders and collagen vascular diseases, have a well-established association with acquired haemophilia A [3]. It is not unexpected that auto-antibodies to FVIII can be found in patients infected with hepatitis C virus, and four cases have been reported. Only one case of HIV/HCV-co-infected patient has been described [4].In this study, we present the case of a HIV-infected patient with FVIII inhibitor. A 65-year-old man was admitted to Emergency Department on May 2008 for the appearance of spontaneous ecchymoses on his trunk and the presence of massive muscle haematomas on the left thigh. The patient also had painless haematuria for 1 week.There was no family or personal history of a congenital bleeding diathesis. The patient was diagnosed with HIV infection in 2003 and was receiving highly active antiretroviral therapy with nevirapine, lamivudine and didanosine. His HIV viral load was 1.60 < log copies mL )1 , with a CD4+ cell count of 564/mm 3 . On physical examination, the patient was afebrile, no lymph nodes were palpable and the liver and spleen were not enlarged.Blood tests showed severe anaemia (haemoglobin 6.6 g dL )1 ) and markedly prolonged activated partial thromboplastin time (APTT; 78 s.). PT, fibrinogen and platelet count were within the normal ranges. The serum creatinine was 2.6 mg dL )1 and liver function was normal. The direct and indirect CoombsÕ tests were negative.Considering the emergency of the initial clinical picture, we decided to start transfusion therapy with red blood cell concentrates (20 units of RBC were transfused during the hospital care) and fresh frozen plasma transfusion immediately. The antiretroviral therapy was promptly stopped.Hepatitis B virus serology showed anti-HBs >1000, with HBsAg, HbeAg and anti-HBe negative. HCV serology was negative. The rheumatoid factor level, the serum immunoglobulin levels, anti-cardiolipin IgG and the anti-cardiolipin IgM were normal. The antinuclear antibody was negative. Examination of malignancies and dermatological disorders, and reaction to drugs were excluded. Abdominal magnetic resonance showed a vast (10 cm) haematoma involving left thoracic and abdominal wall.The laboratory tests showed an extremely low value of FVIII (<1 U dL )1 ) and a high titre of inhibitor (118 BU mL )1 ). The patient was diagnosed as having a coagulopathy caused by an acquired FVIII inhibitor. The patient was started on oral prednisone 80 mg daily and i.v. recombinant human coagulation FVIIa [rFVIIa; NovoSeven (Princeton, NJ, USA); loading dose 90 mcg kg )1 , then 16 m...