Retroperitoneal and compartmental bleeding (retropharingeal, extensive musle haematomata with compression of nervous and vascular structure) are challenging and often misdiagnosed. Haemarthroses are less frequent than in congenital haemophilia: in the Italian series, 7.3% of 96 patients at presentation and 16.1% of 31 patients at relapse . Intracranial bleeding is rare (2% in the Italian survey), but catastophic. Severe bleeding can ensue surgical interventions because of an overlooked prolonged
activated partial thromboplastin time (APTT). In the Italian survey, the preoperative APTT, when carried out, was always prolonged . Anaemia is frequent; its severity is related to the severity of bleeding. PXD101 supplier The diagnosis of acquired haemophilia is easily
suspected when the APTT is prolonged in the proper clinical setting. A prolonged APTT with normal prothrombin time should always arouse suspicion. The APTT is reported as the ratio of patient/normal plasma. The subsequent step is to carry out the mixing test. The APTT is carried out in a mixure (1:1) of normal and patient’s plasma, after incubation for 2 h at 37°C (the interaction inhibitor-FVIII:C is temperature- and time-dependent) with no correction. Further testing of FVIII:C and the inhibitor titre is available only in specialized laboratories. A prolonged APTT is also found in the lupus anticoagulant (LA), the anti phospholipid 上海皓元医药股份有限公司 (APA)
syndromes and during unfractionated heparin PD-0332991 chemical structure therapy. The LA and APA syndromes are acquired thrombophilic factors and heparin treatment is easily ruled out. The objectives of the management are control of bleeding and suppression of inhibitor. The sudden onset of clinically significant bleeding with a prolonged APTT, suggesting acquired haemophilia, requires urgent consultation and transfer of the patient to the reference centre. Control of the acute bleeding is the priority because of the high early mortality. The inhibitor titre and the FVIII:C levels are not correlated with the clinical picture; therefore, being at variance with patients with congenital haemophilia, they are not valuable in guiding therapy . The most significant criteria are clinical: the site and the intensity of bleeding. First-line haemostatic treatment includes recombinant activated FVII recombinant factor VIIa (rFVIIa) and activated prothrombin complex concentrate (APCC). The published studies are retrospective and include a limited number of patients with different primary clinical conditions. No prospective randomized trial comparing their efficacy has been carried out to date as such a study is not practicable. Neither one is effective in every patient. The dosage of these agents is derived largely from the experience of the treatment of congenital haemophilia with inhibitor. No information on duration of treatment is available.