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Clinical and epidemiological research
Concise report
Systemic mastocytosis and bone involvement in a cohort of 75 patients
  1. S Barete1,2,3,
  2. N Assous1,
  3. C de Gennes4,
  4. C Grandpeix3,
  5. F Feger2,
  6. F Palmerini5,
  7. P Dubreuil5,
  8. M Arock6,
  9. C Roux7,
  10. J M Launay3,8,
  11. S Fraitag3,9,
  12. D Canioni3,9,
  13. B Billemont10,
  14. F Suarez11,
  15. F Lanternier3,12,
  16. O Lortholary3,12,
  17. O Hermine2,3,11,
  18. C Francès1,3
  1. 1AP-HP, Dermatology and Allergy Department, Université Pierre et Marie Curie, Tenon Hospital, Paris, France
  2. 2CNRS-UMR 8147, IFR Necker, Necker Hospital, Paris, France
  3. 3Centre de référence des mastocytoses (CEREMAST), Faculté Necker, Paris, France
  4. 4AP-HP, Internal Medicine Department, Université Pierre et Marie Curie, Pitié-Salpêtrière Hospital, Paris, France
  5. 5INSERM, Centre de Recherche en cancérologie, Paoli Calmettes Institute, Marseille, France
  6. 6AP-HP, Biochemical Department, Université Pierre et Marie Curie, Pitié-Salpêtrière Hospital, Paris, France
  7. 7AP-HP, Rheumatology B Department, Université Paris-Descartes, Cochin Hospital, Paris, France
  8. 8AP-HP, Biochemical Department, Université Paris VII, Lariboisière Hospital, Paris, France
  9. 9AP-HP, Pathology Department, Université Paris-Descartes, Necker Hospital, Paris, France
  10. 10AP-HP, Cochin Hospital, CERIA; Université Paris-Descartes, Paris, France
  11. 11AP-HP, Hematology Department, Université Paris-Descartes, Necker Hospital, Paris, France
  12. 12AP-HP, Infectious Disease Department, Université Paris-Descartes, Necker Hospital, Paris, France
  1. Correspondence to Dr Stéphane Barete, Dermatology and Allergy Department, Université Pierre et Marie Curie, Tenon Hospital, 4 rue la Chine, Paris 75020, France; stephane.barete{at}psl.aphp.fr

Abstract

Objectives To investigate bone involvement in a large cohort of systemic mastocytosis (SM) patients, and evaluate the efficacy of bisphosphonate therapy.

Patients and Methods From 2000 to 2004, 75 patients with SM according to WHO criteria underwent skeletal x-rays and bone mineral density (BMD) assessment. Sequential BMD assessments were performed in nine patients treated with bisphosphonate (mean follow-up 65 months).

Results 37 patients (49%) had bone involvement according to both x-rays and BMD evaluations: osteoporosis (23 patients, 31%, mean lumbar spine T score: −3 SD), with vertebral fracture (13 patients, 17%), axial skeleton osteosclerosis (six patients, 8%), mixed patterns (three patients), osteopenia with pre-existing fractures (four patients) and focal osteolytic lesion (one patient). Blood count abnormalities were associated with osteosclerosis (p=0.005). In nine patients with osteoporosis and bisphosphonate therapy, mean lumbar spine BMD increased from 0.83 to 0.92 g/cm2 (+11.1%; ie, +2.05% per year) without recurrence of vertebral fracture.

Conclusion Half of adult patients with SM have bone involvement. Osteoporosis is the most prevalent bone manifestation in SM (31%). Bisphosphonate therapy seems efficient to improve lumbar spine BMD during SM-related osteoporosis. Spine x-ray and BMD should be performed in all SM patients to detect those who may benefit from anti-osteoporotic therapy.

https://doi.org/10.1136/ard.2009.124511

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    Footnotes

    • Funding This study has been supported by grants from GIS maladies rares, AFIRMM (Association Française d'Initiatives pour la Recherche sur les Mastocytes et les Mastocytoses) and Ligue Nationale contre le Cancer.

    • Competing interests None.

    • Patient consent Obtained.

    • Ethics approval This study was conducted with the approval of the CCPPRB No 93-00 du 8 November 2000 Pitié-Salpêtrière Hospital.

    • Provenance and peer review Not commissioned; externally peer reviewed.