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[Albers-Schönberg disease]
G Székely1, V Winkler, G Tarkovács
1Ferencvárosi Egészségügyi Központ, Reumatológia, Budapest.
Abstract:
The authors while describing their patients suffering from osteopetrosis disease, discuss its morphological aspects and possible patho-mechanism. The disease with osteosclerosis can be inherited recessively or dominantly. The recessively inherited type is less frequent and leads to early death due to secondary developing myelofibrosis. The dominantly inherited form is more benevolent, the patients are free of symptoms in half of cases. The patients described by the authors belong to the dominantly inherited type of the Albers-Schönberg disease. One of their patients suffers from rheumatoid arthritis and myelodisplastic syndrome apart from osteopetrosis. Having considered the publications authors have found data based on which the common source and connection of these three diseases can be rendered possible. Analyzing these data they draw attention to the possible pathogenic role of cytokines, first of all of the macrophag colony stimulating factor, moreover to the rheumatic manifestation of the paraneoplastic syndrome.