Abstract
A hemophilic pseudotumor is one of the rarer complications of hemophilia that results from repetitive bleeding resulting in an encapsulated mass of clotted blood and necrosed tissue. These have become rarer over the years with better treatment modalities for bleeding disorders like factor replacement. In this case report, we describe the natural history and clinical course of a patient with hemophilia who presented with a massive pseudotumor. A review of literature follows which explores all the relevant data to date on the presentation, complications, diagnostic modalities and therapies that have been implemented in the management of this rare complication of a common bleeding disorder.
Highlights
A hemophilic pseudotumor is one of the rarer complications of hemophilia that results from repetitive bleeding resulting in an encapsulated mass of clotted blood and necrosed tissue
We describe the natural history and clinical course of a patient with hemophilia who presented with a massive pseudotumor
Hemophilia A and B are X-linked clotting disorders caused by the deficiency of factors VIII and IX respectively
Summary
Hemophilia A and B are X-linked clotting disorders caused by the deficiency of factors VIII and IX respectively. Hemophilia manifests with hemorrhage; spontaneous bleeding into joints and muscles is a feature of severe hemophilia [1,2]. A hemophilic pseudotumor is the result of repeated episodes of bleeding at a bone fracture site or as a result of sub-periosteal hemorrhage or bleeding into a soft tissue. These lesions expand over time, eventually causing symptoms by mass effect. The majority of all reported hemophilic pseudotumors involve the musculoskeletal system. A few intra-abdominal pseudotumors have been reported but these were tumors of the pelvis that had extended into the abdomen [3,4]. Very few retroperitoneal pseudotumors have been reported in literature. We describe a patient with a massive retroperitoneal pseudotumor and review the literature on hemophilic pseudotumors
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