Absence of HLA linkage in a family with osteitis deformans (Paget''s disease of bone)

HLA genotyping was carried out on a two-generation family with familial osteitis deformans. No evidence of major histocompatibility linkage of the disease could be demonstrated.     

Pseudosarcoma in Paget's disease of bone

The appearance of a sarcoma of bone is a well-recognized complication of Paget's disease. The most common type of such a sarcoma is osteosarcoma. Much less common are soft tissue lesions adjoining the pagetic bone that clinically and radiologically simulate sarcoma but histologically represent exaggerated periosteal bone formation as a manifestation of the basic pathologic process. We present a case of a bulky juxtacortical soft tissue mass in the thigh arising from a pagetic femur in a 62-year-old patient with polyostotic Paget's disease that was clinically and radiologically suspected to be a juxtacortical osteosarcoma. Microscopically, the lesion showed features of florid Paget's disease without any evidence of sarcomatous growth. It is important to be aware of this rare manifestation of Paget's disease to avoid unnecessary overtreatment.     

Immunoglobulin abnormalities in Paget's disease of bone.

Several patients with Paget's disease have been reported to have monoclonal IgM proteins in their serum. We have systematically studied 26 patients with Paget's disease severe enough to require diphosphonate therapy. Five of these patients were found to have isolated elevations of serum IgM which were well outside the normal range for their age, sex and race. An additional patient had elevations of both IgG and IgM. These patients seem to represent a subset of Pagetics. They do not differ from the remainder of the group with respect to severity or extent of disease, response to therapy, presence of intercurrent disease or age. They do have a different male to female ratio. These observations suggest that some patients with Paget's disease may have disordered immune regulation or that they may be undergoing a primary response to an agent involved in the aetiology or pathogenesis of the disease.