Prevalence and risk factors for fragility fracture in systemic mastocytosis

Degboé, Yannick; Eischen, M.; Nigon, Delphine; Apoil, Pôl-André; Mailhol, C.; Tournier, É.; Laurent, Césari; Hanssens, Katia; Hermine, Olivier; Paul, C.; Laroche, Michel; Livideanu, Cristina Bulai

doi:10.1016/j.bone.2017.09.005

Cited by 29 publications

(16 citation statements)

References 31 publications

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“…The history of osteoporosis or bone fracture at the time of diagnosis can help physicians to differentiate between isolated CM and SM, considering all other osteoporosis risk factors. The frequency of bone fracture in the present SM cohort (20%) is similar to data reported in literature (16–28%) …”

Section: Discussionsupporting

confidence: 91%

“…Also, we show that the absence of cutaneous KIT mutation and a level of bone marrow tryptase <50 lg/L are strong arguments in favour of isolated CM in adulthood. Osteoporosis and bone fracture can occur in SM patients 14,16,18 but not in isolated CM patients with no osteoporosis risk factors. Moreover, we show that patients with SM can present a rapid reduction in bone mineral density over a period of 12-24 months, suggesting that changes induced by mast cells in SM may be responsible for the occurrence of osteoporosis and its complications.…”

Section: Discussionmentioning

confidence: 99%

“…The frequency of bone fracture in the present SM cohort (20%) is similar to data reported in literature (16-28%). 14,16 In this study, the diagnosis of isolated CM was possible by analyses of skin KIT mutation and bone marrow tryptase level, as only these two parameters being statistically different in the multivariable analysis. If these two parameters are validated in a larger external cohort, they could avoid bone marrow biopsies being performed to diagnose SM in adults.…”

Section: Discussionmentioning

confidence: 99%

“…The osteoporosis risk factors recorded were as follows: ever/ never smoking, self-reported excessive alcohol intake defined as >20 g/day for women and >30 g/day for men, body mass index <19 kg/m², endocrinopathy, early menopause, renal disease, exposure to oral corticosteroids and age over 50 years. 14,16 The laboratory data recorded were as follows: serum and bone marrow tryptase level, the KIT D816V mutation in skin and bone marrow aspirate, and the number of dermal mast cells per 940 field. We also recorded the frequency of cases with a serum level tryptase >20 lg/L and of cases with a bone marrow tryptase >50 lg/L, a cut-off defined in literature as a diagnostic criterion for SM.…”

Section: Data Collectionmentioning

confidence: 99%

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Frequency of isolated cutaneous involvement in adult mastocytosis: a cohort study

Fradet

Negretto

Tournier

et al. 2019

Acad Dermatol Venereol

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Background Mastocytosis is characterized by the accumulation/proliferation of abnormal mast cells. The frequency of isolated cutaneous involvement in adults with mastocytosis has not been fully determined. The main objective of our study was to assess the frequency of isolated cutaneous mastocytosis (CM) in adults with mastocytosis skin lesions. The second objective was to compare the clinical, histological, biological and imaging features in patients with isolated CM and patients with systemic mastocytosis (SM). Methods We included all patients with histology‐proven mastocytosis skin lesions between January 2009 and December 2017. The mastocytosis diagnosis was made according to the international diagnostic criteria. All data were collected from a dedicated specific case report. Results Among 160 patients with mastocytosis skin lesions, 25 patients had isolated CM (15.6%), 105 had SM and 30 (18.7%) patients had undetermined mastocytosis. Skin KIT mutation (OR: 51.9, 95% CI: 3.9–678, P = 0.001) and high bone marrow tryptase (OR: 97.4, 95% CI: 10.3–915, P = 0.001) were strong predictors of SM. The prevalence of osteoporosis was higher in the SM population than in the isolated CM population. Moreover, a decrease in bone mineral density over a short period of follow‐up (1–2 years) was associated with SM. There were no differences between the two groups regarding the frequency of mast cell activation symptoms, the presentation of skin lesions, the number of mast cells in the dermis and the level of serum tryptase. We propose considering the KIT mutation status and bone marrow tryptase levels to aid the diagnosis of isolated CM in adult mastocytosis patients. Conclusion Only a small minority of adults with mastocytosis skin lesions has isolated cutaneous involvement. In 18.7% of mastocytosis cases, even complete workup does not allow for a precise classification of patients.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Data Collectionmentioning

confidence: 99%

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Frequency of isolated cutaneous involvement in adult mastocytosis: a cohort study

Fradet

Negretto

Tournier

et al. 2019

Acad Dermatol Venereol

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“…The existing data on tryptase levels, a marker for MC activity, in SM are inconsistent. Many authors describe increased concentrations, which correlate with reduced bone mass, whereas others report normal levels despite bone loss (148,150,155).…”

Section: The Role Of Mast Cells In Bone Disorders Osteoporosismentioning

confidence: 99%

The Role of Mast Cells in Bone Metabolism and Bone Disorders

et al. 2020

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Mast cells (MCs) are important sensor and effector cells of the immune system that are involved in many physiological and pathological conditions. Increasing evidence suggests that they also play an important role in bone metabolism and bone disorders. MCs are located in the bone marrow and secrete a wide spectrum of mediators, which can be rapidly released upon activation of mature MCs following their differentiation in mucosal or connective tissues. Many of these mediators can exert osteocatabolic effects by promoting osteoclast formation [e.g., histamine, tumor necrosis factor (TNF), interleukin-6 (IL-6)] and/or by inhibiting osteoblast activity (e.g., IL-1, TNF). By contrast, MCs could potentially act in an osteoprotective manner by stimulating osteoblasts (e.g., transforming growth factor-β) or reducing osteoclastogenesis (e.g., IL-12, interferon-γ). Experimental studies investigating MC functions in physiological bone turnover using MC-deficient mouse lines give contradictory results, reporting delayed or increased bone turnover or no influence depending on the mouse model used. By contrast, the involvement of MCs in various pathological conditions affecting bone is evident. MCs may contribute to the pathogenesis of primary and secondary osteoporosis as well as inflammatory disorders, including rheumatoid arthritis and osteoarthritis, because increased numbers of MCs were found in patients suffering from these diseases. The clinical observations could be largely confirmed in experimental studies using MC-deficient mouse models, which also provide mechanistic insights. MCs also regulate bone healing after fracture by influencing the inflammatory response toward the fracture, vascularization, bone formation, and callus remodeling by osteoclasts. This review summarizes the current view and understanding of the role of MCs on bone in both physiological and pathological conditions.

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Systemic Mastocytosis and Bone-Related Events

Asadipooya

Greene

2019

Mastocytosis

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Prevalence and risk factors for fragility fracture in systemic mastocytosis

Cited by 29 publications

References 31 publications

Frequency of isolated cutaneous involvement in adult mastocytosis: a cohort study

Frequency of isolated cutaneous involvement in adult mastocytosis: a cohort study

The Role of Mast Cells in Bone Metabolism and Bone Disorders

Systemic Mastocytosis and Bone-Related Events

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