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Successful treatment of giant abdominal haemophilic pseudotumours causing hydronephrosis
  1. Shuzhong Liu1,
  2. Xi Zhou1,
  3. An Song2,
  4. Zhen Huo3,
  5. Yipeng Wang1,
  6. Yong Liu1
  1. 1Department of Orthopaedic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
  2. 2Department of Endocrinology, Key Laboratory of Endocrinology, National Health and Family Planning Commission, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
  3. 3Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
  1. Correspondence to Professor Yong Liu, Department of Orthopedic Surgery, Peking Union Medical College Hospital, Dongcheng-qu 100730, China; liuyong_pumch{at}163.com

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A 37-year-old man with a 30-year history of haemophilia A presented to our institution with intensifying abdominal pain. The patient gave a history of paroxysmal and severe abdominal pain for approximately 2 years and stated that he had a family history of haemophilia A. No other relevant family or personal history was identified. Ultrasonography demonstrated severe hydronephrosis of the right kidney and a giant 20×15 cm retroperitoneal mass. His medical treatment included administration of recombinant factor VIII concentrate of 800 IU almost once a week for some years and analgesics. On further questioning, the patient stated the haemophilia A had been relatively well controlled on the maintenance dose of recombinant factor VIII prior to his admission. No major bleeding complications had occurred in the 6 months before admission. Laboratory tests revealed normal prothrombin time (11.1 s, normal: 10.4–12.6 s), normal factor VIII activity (103.3%, normal: 50.0%–150.0%), normal haemoglobin (160 g/L, normal: 120–160 g/L), creatinine (71 μmol/L, normal: 59–104 μmol/L) and no acquired antibodies. The patient denied experiencing any other constitutional symptoms and had no history of trauma, use of anticoagulant drugs or surgery during the past few years. A computed tomographic scan of the abdomen also demonstrated severe hydronephrosis of the right kidney and what were presumed to be …

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