Abstract
Introduction: Pseudotumours are rare and serious complications of hemophilia, occurring in approximately 1% to 2% of patients. They are thought to be the result of recurrent hemorrhage into the muscles or periosteal or intraosseous spaces and consist of a tough, fibrous capsule surrounding a chronically enlarged coagulum.
Material and methods: Over a period of 10 years (1995–2004) 585 hemophilia patients were studied retrospectively to identify the cases of pseudotumours. Pseudotumours were confirmed on standard clinical and radiological criteria. The treatment modalities used either alone or in combination were the following: (a) Factor replacement therapy (b) Surgery (c) Radiotherapy (1000–2000cGy over 5–10 days). All patients on radiotherapy also received factor replacement (20–30%). Response criteria were (1) Complete response (CR): complete resolution of swelling clinically and radiologically (2) Partial response (PR): absence of clinical symptoms and > 50% reduction in size, (3) No response: neither CR nor PR.
Results: Twenty-three pseudotumours were identified in 22 patients in the age range of 6–54 years (median age: 14 years). The prevalence of pseudotumor in our hemophilia patients was 3.7%. Sixteen patients were hemophilia A and other 6 were hemophilia B. Hemophilia was classified as: severe in 15, moderate in 6 and mild in one. One of the severe hemophilia A patients had factor VIII inhibitors (64BU/l). History of trauma was present in four patients.
Location of pseudotumors: (A) Limbs: 11 [thigh: 6, tibia*: 1, calcaneum: 2, metacarpal bone: 1, thumb: 1]. (B) Face: 2 [mandible*: 1, Para nasal sinus: 1], (C) Abdominal: 10 [illiopsoas: 7, other retroperitoneal: 3]. One patient* had two pseudotumors simultaneously - one in the mandible and other in the left tibia.
Treatment and response: (A) Abdominal pseudotumors: No radiotherapy was given to any of the abdominal pseudotumors. Two patients (illiopsoas: 1, other retroperitoneal: 1) underwent surgical removal with 60–80% factor replacement for 3 weeks with complete response. Other eight patients received only factor replacement (50–60%) therapy; only 1 achieved PR at 4 weeks. (B) Pseudotumors limbs and face: 10/13 patients (thigh: 4, tibia: 1, calcaneum: 2, thumb: 1, mandible: 1, paranasal sinus: 1) received radiotherapy along with 20–30% factor replacement (mostly FFP) therapy. All 10 patients showed response: CR:-6, PR:- 4. None of our patients showed any significant side effects of radiotherapy. Two patients received only factor replacement therapy without improvement. One patient who had a metacarpal bone pseudotumor along with factor VIII inhibitors did not receive any treatment and was lost to follow up.
Conclusion: Pseudotumours are common in Indian hemophilia patients due to limited availability of factor replacement. Radiotherapy appears a cost effective therapy especially in pseudotumors of limbs. The complications are rare in conventional radiation doses (1000–2000cGy) used for the treatment of pseudotumors.
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