The pseudotumor is a potentially limb and life-threatening condition unique to hemophilia that occurs as a result of inadequately treated soft tissue bleeds, usually in muscle adjacent to bone, which can be secondarily involved. It is most commonly

seen in a long bone or the pelvis. If not treated, the pseudotumor can reach enormous size, causing pressure on the adjacent neurovascular structures and pathologic fractures. A fistula can develop through the overlying skin. Diagnosis is made by the physical finding of a localized mass. Radiographic findings include a soft tissue mass with adjacent bone destruction. A more detailed and accurate evaluation of a pseudotumor can be obtained with CT scan and MRI. Management depends on the site, size, rate of growth, and effect on adjoining structures. Options include factor replacement and monitoring, aspiration, and surgical ablation. buy VX-809 A 6-week course of treatment with

factor is recommended, followed by repeat MRI. If the tumor is decreasing, continue with factor and repeat MRI for three cycles. (Level 4) [[42, 43]] Proceed to surgery if necessary, which will be much easier if the tumor has shrunk. Aspiration of the pseudotumor followed by injections of fibrin glue, arterial high throughput screening embolization, or radiotherapy may heal some lesions. Surgery may be needed for others. (Level 4) [[44, 45]] Surgical excisions, including limb amputations, may be necessary for large pseudotumors, particularly if they erode long bones. Large abdominal pseudotumors present a special challenge in surgical management of hemophilia; surgery must only be performed by teams with experience in hemophilia. Fractures are not frequent Pomalidomide in people with hemophilia, possibly due to lower levels of ambulation and intensity of activities [[46]]. However, a person with hemophilic arthropathy may be at risk for fractures around joints that have significant loss of motion and in bones that

are osteoporotic. Treatment of a fracture requires immediate factor concentrate replacement. (Level 4) [ [46-49]] Clotting factor levels should be raised to at least 50% and maintained for 3–5 days. (Level 4) [[3],[46-48]] Lower levels may be maintained for 10–14 days while the fracture becomes stabilized and to prevent soft tissue bleeding. The management plan should be appropriate for the specific fracture, including operative treatment under appropriate coverage of clotting factor concentrates. Circumferential plaster should be avoided; splints are preferred. (Level 4) [[46]] Compound/infected fractures may require external fixators. [[49]] Prolonged immobilization, which can lead to significant limitation of range of movement in the adjacent joints, should be avoided.