Bone haemophilic pseudotumour of the ulna: A rare complication of haemophilia in a dog

Published on Sep 12, 2017in Veterinary and Comparative Orthopaedics and Traumatology0.877
· DOI :10.3415/VCOT-17-01-0002
A. Decambron3
Estimated H-index: 3
(École nationale vétérinaire d'Alfort),
Mathieu Manassero11
Estimated H-index: 11
+ 2 AuthorsVéronique Viateau14
Estimated H-index: 14
Objectives: This case report describes for the first time a bone haemophilic pseudotumour in a dog. Case description: A seven-month-old German Shepherd male dog was presented with the complaint of a forelimb weight-bearing lameness with major swelling that expanded dramatically after fine needle aspiration. Radiographs showed a large, well-defined ulnar diaphyseal cystic-like osteolytic lesion. Based on prolonged activated partial thromboplastin time (aPTT) and low factor VIII activity, haemophilia A was diagnosed. Bone scintigraphy, computed tomography, magnetic resonance imaging, and histological findings definitely ruled out malignant neoplasia or inflammation and strongly supported a bone haemophilic pseudotumour over an aneurysmal bone cyst. Segmental ulnar resection  and replacement by a polymethylmethacrylate spacer combined with perioperative bleeding management resulted in a successful outcome. Discussion: This case provided evidence that a bone haemophilic pseudotumour may be the sole presenting clinical sign of haemophilia A in dogs. Early diagnosis, based on history and magnetic resonance imaging findings, is imperative for prompt treatment leading to successful outcome. It is challenging as fine needle aspiration or biopsy is contraindicated. As described in humans, surgical excision of the lesion combined with management of severe postoperative bleeding was associated with successful outcome in the present case. Clinical significance: A bone haemophilic pseudotumour should be considered in the differential diagnosis of expanding mass associated with osteolysis, especially in young male dogs. Perioperative monitoring of the bleeding disorder and subsequent FVIII replacement therapy was of paramount importance in the present case.
#1Jintao Fang (Southern Medical University)H-Index: 3
#2Xiaoreng Feng (Southern Medical University)H-Index: 4
Last. Bin Chen (Southern Medical University)H-Index: 10
view all 6 authors...
2 CitationsSource
#1Horacio CavigliaH-Index: 8
#1H. CavigliaH-Index: 3
Last. Gustavo GalatroH-Index: 7
view all 6 authors...
Summary Development of inhibitors against factor VIII (FVIII) or FIX is the most serious complication of replacement therapy in patients with haemophilia. Haemophilic pseudotumours in a patient with inhibitors can lead to devastating consequences. The aim of this study is to show our experience in the treatment of 10 pseudotumours in 7 patients with inhibitors who were treated by the same multidisciplinary team in the period between January 2000 and March 2013. Seven severe haemophilia A patient...
8 CitationsSource
Objective—To evaluate the clinical course of dogs with hemophilia A (factor VIII deficiency) and to determine whether factor VIII coagulant activity (FVIII:C) was associated with severity of clinical signs and outcome. Design—Survey study. Sample—Respondent information for 39 client-owned dogs with FVIII deficiency. Procedures—Information was obtained via a survey distributed to the American College of Veterinary Internal Medicine and American College of Veterinary Emergency and Critical Care em...
5 CitationsSource
#1Soo Fin Low (UKM: National University of Malaysia)H-Index: 5
#2Radhika Sridharan (UKM: National University of Malaysia)H-Index: 6
Last. Nor Hazla Mohamed Haflah (UKM: National University of Malaysia)H-Index: 6
view all 4 authors...
Pseudotumours are rare, occurring in 1–2% of severe haemophiliacs. Osseous locations are far less frequent than soft tissue location. We report a case of a 43-yearold man with haemophilia A, who presented with a gradually enlarging left thigh mass for 8 months. There were no constitutional symptoms. Plain radiograph showed an expansile lytic lesion with ‘soap-bubble’ appearance arising from the left femur diaphysis. On MRI, it appeared as a non-enhancing, multilobulated lesion expanding the medu...
3 CitationsSource
#1Ming Y. Lim (UNC: University of North Carolina at Chapel Hill)H-Index: 9
#2B. NielsenH-Index: 1
Last. Nigel S. Key (UNC: University of North Carolina at Chapel Hill)H-Index: 78
view all 4 authors...
Summary Given the rarity of haemophilic pseudotumours, consensus on management is lacking. We describe the clinical features and management of haemophilic pseudotumours by retrospectively reviewing the medical records of haemophilia patients with a diagnosis of pseudotumour seen at our Hemophilia Center from 1981 to 2011. We recorded the following data: type and severity of haemophilia, documented aetiological antecedent, localization of the pseudotumour, presenting symptoms, management and outc...
23 CitationsSource
#1Joannis Panotopoulos (Medical University of Vienna)H-Index: 11
#2Cihan Ay (Medical University of Vienna)H-Index: 48
Last. Hugo A. Wanivenhaus (Medical University of Vienna)H-Index: 3
view all 10 authors...
Purpose Haemophilic pseudotumour was defined by Fernandez de Valderrama and Matthews as a progressive cystic swelling involving muscle, produced by recurrent haemorrhage into muscles adjacent to the bone. The pseudotumour mainly occurs in the long bones and the pelvis. The treatment of the haemophilic pseudotumour poses a challenge, and extensive clinical experience is essential to appropriately address this serious complication in patients with haemophilia. Consequently, the aim of this study i...
18 CitationsSource
#1Jason S. Pruzansky (ISMMS: Icahn School of Medicine at Mount Sinai)H-Index: 2
#2Marvin S. GilbertH-Index: 1
Last. Richard S. GilbertH-Index: 5
view all 4 authors...
Hemophilic pseudotumors are rare, particularly when they occur in the distal extremity of an adult patient. We present the case of a 68-year-old man with well-controlled factor VIII deficiency who presented with a lytic lesion of the distal radius that was identified as an intraosseous pseudotumor.
6 CitationsSource
#1Jérôme Benamou (MSU: Michigan State University)H-Index: 7
#2Bertrand LussierH-Index: 16
Last. Claudine SavardH-Index: 2
view all 5 authors...
Case Description—An 18-month-old spayed female domestic shorthair cat was evaluated because of left thoracic limb lameness. Clinical Findings—A firm mass was palpable in the left scapular region. On the basis of clinical signs; results of radiographic, ultrasonographic, and cytologic evaluations; and findings on magnetic resonance imaging, an aneurysmal bone cyst (ABC) of the scapula was strongly suspected. Treatment and Outcome—Considering the large size of the mass and the poor prognosis for r...
8 CitationsSource
#1Sriram Jaganathan (AIIMS: All India Institute of Medical Sciences)H-Index: 4
#2Shivanand Gamanagatti (AIIMS: All India Institute of Medical Sciences)H-Index: 24
Last. Ankur Goyal (AIIMS: All India Institute of Medical Sciences)H-Index: 12
view all 3 authors...
The purpose of this pictorial assay is to demonstrate the imaging features of the various musculoskeletal manifestations of hemophilia, an X-linked disorder. Depending on the site of recurrent bleeding, musculoskeletal manifestation can be in the form of hemophilic arthropathy and/or soft tissue, intraosseous, or subperiosteal pseudotumors. Radiography, sonography, computed tomography, and especially magnetic resonance imaging help in the evaluation of hemophilic arthropathy and pseudotumors, pr...
11 CitationsSource
#1Ramin Espandar (Tehran University of Medical Sciences)H-Index: 8
#2Pedram Heidari (Tehran University of Medical Sciences)H-Index: 17
Last. E. C. Rodriguez-Merchan (Hospital Universitario La Paz)H-Index: 36
view all 3 authors...
Summary. The haemophilic pseudotumour is an expanding destructive haematoma, which is associated with a considerable amount of morbidity in haemophilic patients. Its prevention is paramount. In fact, this goal can be achieved by primary prophylaxis to avoid muscle haematomas and by adequate and long-term haematological treatment of muscle haematomas in case they appear. At the moment, surgical excision of pseudotumour is the preferred treatment by many authors. However, there are instances that ...
55 CitationsSource
Cited By0