A Rapid Skeletal Turnover in Radiographic Mimic of Osteopetrosis Might Be Secondary to Systemic Mastocytosis

Rossini, Maurizio; Viapiana, Ombretta; Adami, Silvano; Idolazzi, Luca; Zanotti, Roberta; Gatti, Davide

doi:10.1002/jbmr.2456

Cited by 5 publications

(2 citation statements)

References 7 publications

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“…Furthermore, in SM patients, focal osteosclerotic and osteolytic bone lesions can often coexist [67]. In these patients a higher bone turnover has been found in both osteoporosis and osteosclerosis [68]; therefore, fractures can be associated with both of these disorders. In addition, the level of tryptase may predict mast cell mass and simultaneously best correlate to higher bone density; however, this high bone density does not protect against fracture in SM patients [69].…”

Section: Bone Involvementmentioning

confidence: 99%

Hematological Diseases and Osteoporosis

Gaudio

Xourafa

Rapisarda

et al. 2020

IJMS

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Secondary osteoporosis is a common clinical problem faced by bone specialists, with a higher frequency in men than in women. One of several causes of secondary osteoporosis is hematological disease. There are numerous hematological diseases that can have a deleterious impact on bone health. In the literature, there is an abundance of evidence of bone involvement in patients affected by multiple myeloma, systemic mastocytosis, thalassemia, and hemophilia; some skeletal disorders are also reported in sickle cell disease. Recently, monoclonal gammopathy of undetermined significance appears to increase fracture risk, predominantly in male subjects. The pathogenetic mechanisms responsible for these bone loss effects have not yet been completely clarified. Many soluble factors, in particular cytokines that regulate bone metabolism, appear to play an important role. An integrated approach to these hematological diseases, with the help of a bone specialist, could reduce the bone fracture rate and improve the quality of life of these patients.

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Section: Bone Involvementmentioning

confidence: 99%

Hematological Diseases and Osteoporosis

Gaudio

Xourafa

Rapisarda

et al. 2020

IJMS

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“…In , the title of the Letter to the Editor was incorrectly printed as “A Rapid Skeletal Turnover in Radiographic Mimic of Osteopetrosis Might Be Secondary to Systemic Mastocytosis.” The correct title reads “Rapid Skeletal Turnover in a Radiographic Mimic of Osteopetrosis Might Be Secondary to Systemic Mastocytosis.”…”

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confidence: 99%

Letter to the Editor: Rapid Skeletal Turnover in a Radiographic Mimic of Osteopetrosis Might Be Secondary to Systemic Mastocytosis

Rossini¹,

Viapiana²,

Adami³

et al. 2015

Journal of Bone and Mineral Research

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Dickkopf-1 and Sclerostin Serum Levels in Patients with Systemic Mastocytosis

et al. 2015

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Bone involvement, mainly osteoporosis but also osteosclerosis, is frequent in patients with indolent systemic mastocytosis (ISM). The recent characterization of the canonical Wnt/β-catenin pathway in the regulation of bone remodeling provided important insights for our understanding of the pathophysiology of a number of conditions. The regulation of Wnt pathway in bone is predominantly driven by the production of receptor inhibitors such as Dickkopf-1 (DKK1) and sclerostin (SOST). This study aimed to explore if the various bone involvements in patients with ISM might be explained by variations in serum levels of DKK1 and SOST. This is a cross-sectional study in an adult ISM cohort (13 men and 13 women with diagnosed ISM) and fifty-two healthy sex and age-matched controls. Early morning, fasting and venous sampling was obtained in all subjects. The main outcome measures were serum bone-specific alkaline phosphatase (bALP), C-terminal telopeptides of type I collagene (CTX), DKK1, SOST, parathyroid hormone (PTH), bone mineral density, and prevalent vertebral fractures. Mean DKK1 serum levels were about two-folds higher in patients, than in controls (65,0 ± 43.3 vs. 33.1 ± 19.4 pmol/L, respectively; p < 0.001), irrespective of the presence of osteoporotic or diffuse osteosclerotic bone involvement. DKK1 serum levels were positively correlated with PTH and both CTX and bALP. Mean SOST serum levels were not significantly different in patients versus controls, and we did not observe any significant correlation between SOST and any available clinical or laboratory parameters, with the only exception of a positive correlation with age. In conclusion, in our study, we observed that DKK1, but not SOST, serum levels significantly increased in ISM patients with various bone involvements, and correlated with PTH and bone turnover markers. Our results suggest that the Wnt/β-catenin pathway is not primarily involved in the pathophysiology of the array of bone involvement in ISM.

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A Rapid Skeletal Turnover in Radiographic Mimic of Osteopetrosis Might Be Secondary to Systemic Mastocytosis

Cited by 5 publications

References 7 publications

Hematological Diseases and Osteoporosis

Hematological Diseases and Osteoporosis

Letter to the Editor: Rapid Skeletal Turnover in a Radiographic Mimic of Osteopetrosis Might Be Secondary to Systemic Mastocytosis

Dickkopf-1 and Sclerostin Serum Levels in Patients with Systemic Mastocytosis

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