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Bone complications of mastocytosis: a link between clinical and biological characteristics.

The American journal of medicine (2012-12-04)
Nicolas Guillaume, Judith Desoutter, Olivia Chandesris, Lavinia Merlusca, Isabelle Henry, Sophie Georgin-Lavialle, Stéphane Barete, Isabelle Hirsch, Daoud Bouredji, Bruno Royer, Bérengère Gruson, Catherine Lok, Henri Sevestre, Romuald Mentaverri, Michel Brazier, Jonathan Meynier, Olivier Hermine, Jean-Pierre Marolleau, Said Kamel, Gandhi Damaj
ZUSAMMENFASSUNG

Mastocytosis is a heterogeneous group of clonal mast cell disorders in which bone manifestations are frequently seen, but poorly understood. In this study, we analyzed correlation of clinical findings in mastocytosis patients with bone mineral density and bone turnover markers. Serum levels of bone turnover markers were measured in mastocytosis patients and healthy volunteers. Bone disease was evaluated using radiographic imaging, and measurement of bone mineral density. Of 45 adult mastocytosis patients, bone abnormalities were detected in 34 (75%). Bone lesions were documented on radiographic imaging in 16 patients (36%), and bone mineral density in 24 patients (53%), of which 9 patients (20%) had osteoporosis and 15 (33%) had osteopenia. Serum levels of bone turnover markers that evaluate bone resorption (C-telopeptide, deoxypyridinoline), bone formation (bone-specific alkaline phosphatase), and bone remodeling (osteoprotegerin) were significantly higher in the patient population than in the control population (n=28). Levels of C-telopeptide and osteoprotegerin were higher in patients with advanced systemic mastocytosis than in patients with cutaneous or indolent systemic mastocytosis. Moreover, C-telopeptide and osteoprotegerin levels were significantly correlated with those of serum tryptase, a diagnostic marker of mastocytosis. The observed bone turnover markers variations indicate a complex process of bone turnover in mastocytosis-related bone manifestations. The highly significant correlation between serum tryptase and serum bone turnover markers levels, and the positive correlation of levels of bone turnover markers with advanced disease, support the existence of a link between bone remodeling and mast cell burden.