Treatment of pelvic haemophilic pseudotumour: A retrospective study

Haemophilia. 2020 Nov;26(6):e308-e314. doi: 10.1111/hae.14148. Epub 2020 Nov 3.

Abstract

Background: The incidence of a pelvic haemophilic pseudotumour is very low and is rarely seen in the clinic. Due to the lack of clear treatment standards, patients often suffer from the condition over a protracted period. The aim of this retrospective study was to present our institutional experience in the treatment of pelvic haemophilic pseudotumours over the past 8 years.

Methods: We retrospectively analysed patients with a pelvic haemophilic pseudotumour who were treated in the Nanfang hospital between February 2012 and December 2019. The type and severity of haemophilia, the presence of inhibitors, comorbidities, pseudotumour imaging data, treatment and follow-up results were recorded and analysed.

Results: Pelvic pseudotumours were identified in seven patients with haemophilia. Three patients had severe haemophilia, three had moderate haemophilia, one had mild haemophilia and inhibitors were present in two patients. Transfusion-related infectious diseases were noted in three patients. Spontaneous rupture and infection of the pseudotumour occurred in five patients. In addition, five patients underwent surgical treatment, two of whom healed well, two patients suffered recurrence of the pseudotumour, and one patient developed a postoperative haematoma twice. Two patients were treated conservatively, one of whom was unable to walk because of progression of the disease, while the other died from severe bleeding and infection.

Conclusions: Once a pelvic haemophilic pseudotumour is diagnosed, surgical resection should be performed as soon as possible. A delay in diagnosis and suboptimal treatment may lead to complications of the pelvic haemophilic pseudotumour.

Keywords: haemophilia; immune tolerance; infection; inhibitors; pelvic pseudotumour; treatment.

MeSH terms

  • Adult
  • Hemophilia A / complications*
  • Hemophilia A / therapy*
  • Humans
  • Incidence
  • Middle Aged
  • Pelvis / pathology*
  • Pseudotumor Cerebri / therapy*
  • Retrospective Studies
  • Young Adult