Paget Disease of the Bone Flashcards
What is Paget disease?
Disordered bone remodeling in which excessive bone resorption initially results in lytic lesions followed by disorganized and excessive bone formation
What is the pathologic cell of Paget disease?
Osteoclast
Describe the pathogenesis of Paget’s Disease of the bone.
- Widespread osteolysis due to overactivation of osteoclasts
- Mixed osteoblastic and osteoclastic activity occurs producing larger than normal bones
- Cold (burnt-out stage) little cellular activity and thickened and disordered bones
What is the sign seen on Xray caused by the cortical resorption that occurs in Paget Disease of the bone?
Flame sign
What is observed on microscopy of the osteoclast?
Many more nuclei than a usual osteoclast
What is the Dx hallmark of Paget disease of the bone?
Disorganized lamellar bone that has prominent cement lines and mosaic pattern.
Term used for localized lysis in the frontal and parietal bones of the skull?
Osteoporosis circumscripta
In PDB, what is the term used for the flattening of the base of the skull and the impingment on the foramen magnum, thereby compressing the medulla and upper spinal cord?
Platybasia
What is the term of increased size in maxillary bones resulting from PDB?
Leontiasis ossea
What is pagetic steal?
Where blood is shunted to the bones inseated of the carotid system
Complications of PDB?
- Fractures and arthritis
- High output Cardiac failure
- Sarcomatous change (usually osteogenic)
What products are elevated in the serum of a patient with PDB?
Hydroxyproline and hydroxylysine
What is the most useful laboratory test in Dx PDB?
Serium alkaline phosphatase level (gauge of osteoblast activity)
