Hemophilic pseudotumor: Multicenter experience over 25‐year period

Abstract

Pseudotumor is an infrequent manifestation of bleeding diathesis seen in hemophiliacs, the management of which is still controversial. To ascertain the effectiveness of the main therapies of choice, we have reviewed 1,831 patients affected by hemophilias A (1,108) and B (172), von Willebrand's disease (329), and other miscellaneous coagulopathies (222) diagnosed between 1965 and 1990 in a multicentric, retrospective study. Pseudotumor was proven in 21 patients. Replacement therapy was given in 15 cases as the first therapeutic approach, with complete success attained in only two, whereas surgery, which was carried out in 14 patients, completely resolved the process in eight (P = 0.017). Patients over 40 years of age demonstrated worse prognosis than younger patients (P = 0.02), but no other clinical parameters were shown to have influenced the patients' evolution. Surgical management was the most effective treatment for pseudotumor, although more conservative therapies cannot be overlooked in selected cases.

Surgical treatment of the haemophilic pseudotumour: A single centre experience

“…Apart from the multicentre study of Magallon et al [11] only one comparatively large series of analyses [18] on the surgical treatment of haemophilic pseudotumours has been reported. To the best of our knowledge, our current report presents one of the largest groups of patients with severe haemophilia in whom surgery was performed for haemophilic pseudotumours in a single center.…”

“…Different therapy options have been described in the treatment of the haemophilic pseudotumour including surgical resection, arterial embolisation, radiotherapy, percutaneous curettage, and filling with fibrin and/or bone graft or hydroxyapatite [2,3,[10][11][12][13][14][15][16][17]. However, treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia.…”

Pseudotumor of the Mandible as First Presentation of Hemophilia in a 2-Year-Old Male: A Case Report and Review of Jaw Pseudotumors of Hemophilia

“…Hemosiderin and hemosiderinladen macrophages are frequently seen, as evidence of chronic bleeding. When bone is involved, both medullary and cortical destruction is common and necrosis may be seen in adjacent tissues [21]. Calcification and reactive peripheral bone formation may be seen in lesions over time.…”

“…The most comprehensive review of PTH came from a Spanish multicenter experience over a 25-year period [21]. Of 1,831 total patients with coagulopathies, 21 patients (0.01%) (age range: 8-61; median 40) were found to have PTHs.…”

Apresentações incomuns no diagnóstico por imagem do pseudotumor intraósseo do hemofílico

“…Because of bleedings in different times, the hemophilic pseudotumor may present irregular septations and even solid areas of organization, with fibrocicatricial tissue that may present enhancement after intravenous contrast injection (9) . The radiologist and the assisting physician must have knowledge of these presentations, albeit uncommon, in order to perform an appropriate evaluation and avoiding unnecessary biopsy, obviously undesirable in a hemophilic patient, because of the high incidence of complications, including bleedings, fistula and infection (10) . The heterogeneous aspect of the lesion contents at MRI is caused by the different stages of bleeding and blood degradation.…”