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Endocrine Abstracts (2015) 37 EP303 | DOI: 10.1530/endoabs.37.EP303

1Endocrine Unit, 2nd Department of Internal Medicine–Propaedeutic, ‘Attikon’ University Hospital, Athens, Greece; 22nd Department of Internal Medicine–Propaedeutic, ‘Attikon’ University Hospital, Athens, Greece; 3Mastocytosis Outpatient Clinic, Allergy Unit ‘D. Kalogeromitros’, 2nd Department of Dermatology and Venereology, ‘Attikon’ University Hospital, Athens, Greece; 42nd Department of Dermatology and Venereology, ‘Attikon’ University Hospital, Athens, Greece.


Introduction: Systemic mastocytosis (SM) is a rare disorder characterised by clonal proliferation of abnormal mast cells in several tissues, most often skin and bone marrow. Indolent systemic mastocytosis (ISM) is the commonest disease variant in adults, characterised by very low rate of mast cell proliferation. SM has been recognised as a cause of secondary osteoporosis.

Objective: To evaluate bone mineral density and fragility fractures in ISM patients.

Methods: Fourteen patients (nine women and seven premenopausal), aged 27–63 years (43.4±11.8) diagnosed according to World Health Organization criteria were studied retrospectively. Clinical and biochemical data and bone mineral density (BMD) measurements by dual X-ray absorptiometry at the lumbar spine, the total proximal femur and the lower one-third radius were analysed. T-score was used to define osteopenia (<−1 to >−2.5 S.D.) or osteoporosis (−2.5 S.D. or lower) in postmenopausal women or men older than 50 years, and z-score <−2.0 for low BMD in younger men and premenopausal women, according to the International Society for Clinical Bone Densitometry. No patient reported other diseases or use of treatments known to affect bone or mineral metabolism, at initial assessment.

Results: Two patients (14.3%) had osteoporosis, two patients (14.3%) had osteopenia and seven patients (50%) had low BMD. BMD was generally lower at the spine than at the hip. Three patients (21.4%) reported fragility fractures: a 43-year-old premenopausal woman and a 38-year-old man had vertebral fractures, while a 31-year-old premenopausal woman had non-vertebral fractures. None of the patients with fragility fractures had cutaneous mastocytosis and only one of them reported a mild episode of anaphylaxis.

Conclusion: Bone involvement is frequent in ISM patients and may be the initial manifestation. Osteoporotic fractures of unknown aetiology should lead to the suspicion of SM particularly in individuals younger than 50 years.

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