Bone Complications of Mastocytosis: A Link between Clinical and Biological Characteristics

Bone morbidity in chronic myeloproliferative neoplasms

“…It is worth noting that osteoporosis and fractures were observed in male and female patients younger than 50 years, an age group in which osteoporosis is generally very uncommon [8]. In a Swedish cross-sectional study including 16 patients with varying degrees of mastocytosis, vertebral fractures were found in 13 of 16 patients and observed different types of skeletal lesions, including both low bone mineral density (BMD) as well as increased BMD with Quantitative histomorphometry, assessment of number of osteoblasts and osteoclasts [12] Guillaume et al (2013) Cross-sectional study 45 Radiographic imaging, bone mineral density, bone turnover marker [13] Cundy et al (1987) Case report 1 Osteopenia and response to treatment with inhibition of bone resorption [14] Johansson et al (1996) Cross-sectional study 16 Bone density, bone markers and radiological features [15] Brockow et al (2005) Cross-sectional study 29 Level of interleukin-6 level reflects severity of disease [16] Theoharides et al (2002) Cross-sectional study 26 Level of interleukin-6 level reflects severity of disease and osteoporosis [17] Barete et al (2010) Cross-sectional study 75 Bone involvement assessed by X-ray and DXA [18] Rossini et al (2011) Cross-sectional study 82 Bone mineral density, bone turnover markers and fractures [19] Broesby-Olsen et al (2013) Cross-sectional study 48 KIT D816 mutation burden does not correlate to severity of bone loss in SM [20] Essential thrombocythemia and polycythemia vera Histomorphometry and micro-CT [26] Guermazi et al (1999) Review Images of radiological abnormalities in MF, incl. Osteosclerosis [27] Mellibovsky et al (2004) Case report 9 DXA, bone histomorphometry in MF [28] Diamond et al (2002) Case report and review 4 DXA, bone histomorphometry in MF [29] Farmer et al (2015) Cross-sectional study 18 Bone structure assesses by DXA, HR-pQCT, and bone turnover [30] Barosi et al (1989) Cross-sectional study …”

“…Bone involvement, including not only osteoporosis and osteolytic lesions but also osteosclerosis, has been described in several studies of patients with SM [8, [12][13][14][15][16][17][18][19].…”

How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage)

“…73,74 Studies of markers of bone turnover suggest increased bone remodeling and correlation with serum tryptase in some studies but not others. 75,76 Early institution of antimediator therapy may reverse some diseaserelated bone changes; 77 however, there are no definitive studies in this regard. Bisphosphonate therapy is effective in increasing vertebral bone mineral density (BMD; ;2% per year) 71 and preventing osteoporotic fractures, but has considerably less efficacy in increasing hip and femoral neck BMD.…”

Targeting gene expression during the early bone healing period in the mandible: A base for bone tissue engineering

“…Another study documented a highly significant correlation between serum levels of bone turnover markers and serum tryptase as well as a positive correlation of serum levels of bone turnover markers with the advanced stage of systemic mastocytosis. It was concluded that bone remodeling and mast cell burden are directly related (Guillaume et al, 2013). The down-regulation of mast cell-related genes in the early healing phase in our study might indicate that physiological bone healing also is rather dependent In summary, this transcriptome analysis, with the exemplary depiction of five genes, demonstrates and underlines the great complexity of biological processes during the early bonehealing phases.…”