Grardel B, Hardouin P
Institut Calot. Berck/mer.
Rev Rhum Mal Osteoartic. 1992 Jan;59(1):57-63.
Thirty two cases of the association of mastocytosis and bone lesions were collected in a multicentre study. Five cases involved osteocondensation forms. However, most often (27 cases), there was osteoporosis (OP). The diagnosis was made in the absence of obvious risk factors, and thus often in men (2/3 of patients), when there was the association of pigmented urticaria and an excess of mast cells in bone biopsies. Laboratory, radiological and isotope scan findings are often non-specific, being identical to those encountered in common OP. The histomorphometric profile involves an association of decreased cancellous bone volume, increased area of resorption and decreased bone formation parameters. Progression to malignant mastocytosis occurs essentially in diffuse osteocondensation forms and is rare in OP types. Emphasis must be placed on the importance of qualitative study of bone marrow, using specific stains, since the diagnosis may be missed in the absence of typical skin lesions. Conversely, since a simple increase in mast cell count is possible during common OP, a search for mast cell nodules is important in order to establish the diagnosis with certitude.
在一项多中心研究中收集了32例肥大细胞增多症与骨病变相关的病例。其中5例涉及骨致密化形式。然而,最常见的情况(27例)是骨质疏松症(OP)。诊断是在没有明显危险因素的情况下做出的,因此通常发生在男性患者中(占患者的2/3),此时存在色素性荨麻疹以及骨活检中肥大细胞增多的情况。实验室、放射学和同位素扫描结果通常不具有特异性,与常见OP中所见的结果相同。组织形态计量学特征包括松质骨体积减少、吸收面积增加以及骨形成参数降低。进展为恶性肥大细胞增多症主要发生在弥漫性骨致密化形式中,在OP类型中很少见。必须强调使用特异性染色对骨髓进行定性研究的重要性,因为在没有典型皮肤病变的情况下可能会漏诊。相反,由于在常见OP期间肥大细胞计数可能单纯增加,因此寻找肥大细胞结节对于明确诊断很重要。