Sclerosis of bones in Gaucher's disease
Windholz, F.; Foster, S.E.
American Journal of Roentgenology and Radium Therapy 60(2): 246-250
1948
ISSN/ISBN: 0092-5632 PMID: 18878279 Document Number: 14823
Attention is called to the frequent presence of roentgenological signs of bone formation in the medullary cavity in cases of Gaucher's disease. The new formation of osseous tissue is not excessive and is similar in appearance to regenerative ossification in the surroundings of inflammatory lesions. They occur, however, not only in bone structures adjoining absorptive changes but also in such parts of the medullary cavity in which no roentgenological signs of bone destruction are exhibited. In two of our observations bone cylinders were present and occupied the peripheral portions of the medullary cavity in the proximal two-thirds of the femur. Bone tissue is laid down in such cases in intercellular fibrous structures of Gaucher proliferations as a product of direct metaplasia of collagenous fibers. Such fibers are in abundance in late stages of the disease. Consequently, the presence of ossifications in the medullary cavity or in spongy areas indicates localized or general ageing of the pathological tissues. In early manifestations no appreciable amount of fibers is formed. Therefore, pathological osteogenesis may be absent. Roentgen signs of pathological ossification in Gaucher's disease may be of diagnostic significance. This occurs if newly formed bone tissue is laid down in areas situated about absorptive lesions and involves simultaneously more remote areas of the medullary cavity in which no destructive changes are evident.
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