Prophylactic preparation and surgical extirpation of a very large abdominal blood cyst in a severe haemophilia A patient with inhibitors managed by rFVIIa

Dear Editor,The development of destructive haemophilic cysts(pseudotumours) occurs in approximately 1% ofpatients with severe haemophilia [1]. The recurrentbleeding can cause an encapsulated haematoma,most commonly in the pelvic bones, femur, tibia,small bones of the hand and soft tissues. Destruc-tive haemophilic cysts are typically slow growingand asymptomatic; however, in some cases, surgicalintervention is necessary to prevent progressiveenlargement and potential complications. For exam-ple, bone fractures, massive haemorrhage, infectionand compression, and eroding of adjacent struc-tures [2].Surgeryinpatientswithhaemophiliaischallenging,and for those who develop inhibitors to replacementfactors,successful surgical interventionis even harderto achieve. The development of bypassing agents,such as recombinant activated factor VII (rFVIIa) hasgreatly improved the treatment options available toinhibitor patients; for example, allowing patients toundergo elective orthopaedic surgery despite the risksof bleeding or thromboembolic events [3]. Since1998, the Institute of Hematology and Blood Trans-fusion has gained wide experience with rFVIIa in thetreatment of on-demand bleeding and minor surgeryin patients with haemophilia and inhibitors.We report here the removal of an abdominal bloodcyst in a patient with severe haemophilia A andinhibitors. Haemostatic control was effectively main-tained both before and during surgery with rFVIIa.A 51-year-old haemophilia A patient was diag-nosed with haemophilia A at 3 months of age (FVIIIlevels below 1%, nonsense mutation in exon 24)when massive bleeding occurred following herniec-tomy. After this initial bleeding episode, the patientwas repeatedly hospitalized for bleeding into hisjoints (knees and elbows), haematuria, and forbleeding into the tonsils during tonsillitis. Duringthis period, the patient was treated with cryoproteinfollowed by plasma-derived FVIII concentrates,including the period prior to detection of FVIIIinhibitors.Inhibitors to FVIII were first detected in 1983when the patient was aged 28 years. Despite immu-nosuppressive therapy with corticosteroids andcyclophosphamide the inhibitors remained, withtitres oscillating between 1 and 54 BU mL