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- haemophilic pseudotumour
- definite diagnosis
- surgical treatment
- perioperative management
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 …
Contributors SL wrote the first draft of the manuscript and submitted the manuscript. XZ, AS and ZH were in charge of the patient and collected the data, SL and YL did the follow-up of the patient, and YW and YL critically revised the manuscript. YW and YL contributed equally to this paper. All authors approved the final version. All the listed authors contributed significantly to the manuscript preparation.
Funding This study was funded by Peking Union Medical College Graduate Student Innovation Fund.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.