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Osteopetrosis

Defective Vitamin D Binding Protein is connected to Osteopetrosis. It is logical that a person with a defective GcProtein might also be prone to immune diseases and cancer. There are only a couple of cases published connectng osteopetrosis to cancer, but given the rarity of the disease and the poor prognosis (If untreated, infantile osteopetrosis usually results in death by the first decade of life due to severe anaemia, bleeding or infection. Source) we'd not expect too many cases. Here is some information on osteopetrosis, cancer and immunosuppression. It is interesting to note that the disease can be initiated by viral infection.

 

Small Cell Lung Cancer In A Young Patient With Osteopetrosis Tumori, 92: 563-566, 2006.
Case report: We reported the case of a young man of 31 years affected by osteopetrosis in which a small cell lung cancer developed.

Esophagectomy and splenectomy in a patient with osteopetrosis
Samantha K. Hendren, et.al. J Thorac Cardiovasc Surg 2005;129:1457-1458
"Osteopetrosis is a rare genetic disorder characterized by a failure of osteoclast-mediated bone resorption and by hematologic abnormalities. A patient with osteopetrosis presenting with a distal esophagus cancer and secondary hypersplenism was surgically treated. "

Retroviruses, immunosuppression and osteopetrosis. Labat ML. Biomed Pharmacother. 1986;40(3):85-90.
"Since decades, retroviruses are known to induce the formation of dense bones (osteopetrosis) in animals, either by increasing osteoblastic proliferation (ALVs viruses) or by decreasing osteoclastic bone resorption (FeLV virus). The latter is a good model of the inherited disease since neonatally infected cats die from wasting disease, like mutant op/op rats and children suffering from juvenile-malignant osteopetrosis. These similarities have prompted this review of retrovirus-induced osteopetrosis in animals. We suggest the possibility that retroviruses might be involved, at least in part, in the induction of the human disease."

Characterization of the immunosuppression accompanying virus-induced avian osteopetrosis. R E Smith and L J Van Eldik. PMCID: PMC422178. Infect Immun. 1978 November; 22(2): 452–461.

Abstract: Infection of chickens with a myeloblastosis-associated virus which induced a high incidence of osteopetrosis was accompanied by immunosuppression. The immunosuppression was manifested in the following ways. The weight of the bursa, spleen, and thymus was depressed in infected chickens. Infected animals had a diminished capacity to form hemolytic plaques in a direct assay. Spleen cells from osteopetrotic animals did not respond to phytohemagglutinin, and the spleen and bursa had a decreased proportion of cells possessing surface immunoglobulin. Osteopetrotic animals failed to show an age-dependent increase in the proportion of cells demonstrating surface immunoglobulin that was observed in normal animals. However, several individual chickens with heavy osteopetrosis responded to antigenic stimulation in a normal fashion, indicating that although immunosuppression usually accompanies avian osteopetrosis, it may not contribute directly to abnormal bone proliferation.

 

Retrovirus-induced osteopetrosis in mice. Effects of viral infection on osteogenic differentiation in skeletoblast cell cultures. J. Schmidt, M. Casser-Bette, A. B. Murray, A. Luz, and V. Erfle Am J Pathol. 1987 December; 129(3): 503–510.

 

 

 


 

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