Paget s disease and extramedullary hematopoiesis: A case report

Clinical History

20 year-history of Paget s Disease.Since few weeks cough and fever

Imaging Findings

A 75 year-old man with a 20 year-history of Paget s Disease, insufficiency of the aortic valve and mild systolic hypertension. Since few weeks cough and fever. No other symptoms or biological alterations were associated. The hemoglobin level was normal as the red and white blood cell count as well as the serum level of acid phosphatase and calcium.

Discussion

Paget's disease is a progressive bone disease, monostotic or polyostotic, characterized by hypertrophy of affected bones and accelerated disorganized bone remodeling. It results in bone deformities and pain, with a risk for articular and neurological complications, and fractures. Paget s diseases has unknown etiology (probable viral etiology). It occurs predominantly in males and may involve almost any bone of the body (most frequently skull, vertebrae, hip, femur, knee, ribs and shoulder). The risk of complications, and thus the therapeutic decision, are age related. Bisphosphonates are first-line therapy for Paget's disease . The altered architecture of the bone leads to a softening of the bone itself which commonly results in pathologic fractures. These fractures are usually incomplete and may occur without trauma mainly during the lytic phase of the disease. Prolonged weight bearing can produce loss of height of the vertebral bodies or a compression fracture (maily at the thoraco-lumbar spine). The patient performed a CT scan of the lungs for his pulmonary symptoms. CT showed total involvement of the bones of the thorax by Paget s disease. As an additional finding a mass was found in the right paraspinal region next to the bones. The density of the mass revealed fat content. That probably was a locus of extramedullary hematopoiesis, but the diagnosis is largely presumptive because we haven't got the biopsy proof . In fact softening of bone and the occurrence pathologic fractures may result in extrusion of hematopoietic marrow into paraspinal region, later developing into a locus of active extramedullary hematopoiesis.

Differential Diagnosis List

Final Diagnosis

Paget s disease and extramedullary hematopoiesis

URL:	https://www.eurorad.org/case/690
DOI:	10.1594/EURORAD/CASE.690
ISSN:	1563-4086