Successful treatment of non‐invasive bladder tumour in a haemophilia A patient with high‐responding inhibitors: a case report

Abstract

Patients with haemophilia A and inhibitors have a high risk of bleeding complications during surgery. There are few reports of urological surgery in haemophilia patients [1]. Generally, transurethral resection of bladder tumour (TUR-Bt) is the first choice for patients with non-invasive tumours [2]. However, it is difficult to obtain adequate haemostasis in bladder surgery compared with other surgeries because of the moist environment and the bladder's elasticity. Patients with high-responding inhibitors have an especially high risk of haemorrhage. Orthopaedic surgery for haemophilia patients with high-responding inhibitors has been frequently reported. But there are few reports of any other surgeries. Successful surgeries in haemophilia patients with high-responding inhibitor were reported, such as sigmoid colectomy, nephrectomy, splenectomy [3] and liver resection [4]. This is the first report of TUR-Bt in a haemophilia patient with high-responding inhibitors.A 63-year-old man with severe haemophilia A and high-responding inhibitors, who tested hepatitis C positive and human immunodeficiency virus negative, was admitted to our hospital complaining of gross haematuria. The patient developed high factor VIII (FVIII) inhibitor titres at 37 years of age, and his maximum titre was 84.2 BU mL À1 and last titre was 4.3 BU mL À1 . Coagulation studies revealed a prolonged activated partial thromboplastin time (aPTT) of 113.4 s (range, 25-37 s). The level of FVIII activity was <0.01 IU mL À1 and FVIII inhibitor was 2.7 BU mL À1 . Cystoscopy revealed a non-invasive papillary bladder tumour (Fig. 1), but we did not obtain a biopsy specimen because of the patient's bleeding tendency. The patient was diagnosed with a 15 9 20 mm cT1N0M0 bladder tumour, according to computed tomography and magnetic resonance imaging (Fig. 2). After providing informed consent, the patient underwent conventional TUR-Bt. Based on his low inhibitor titre, neutralization therapy using plasma-derived FVIII concentrate (pdFVIII; CROSS EIGHT M â , Japanese Red Cross Society, Chitose, Hokkaido, Japan) was administered at a high dose for surgery. A bolus dose of pdFVIII at 150 IU kg À1 was administered, after which a continuous infusion of FVIII at 4 IU kg À1 h À1 was started. The continuous infusion was sustained for 7 days. Pathological findings revealed urothelial carcinoma, G2, pTa, with complete resection.The patient's Foley catheter was removed on the fourth postoperative day (POD). We suggested urination every 2 h, to avoid bladder expansion. There were no episodes of gross haematuria and the patient