title: Systemic mastocytosis and bone involvement in a cohort of 75 patients.
abstract: OBJECTIVE
  To investigate bone involvement in a large cohort of systemic mastocytosis (SM) patients, and evaluate the efficacy of bisphosphonate therapy.
  
  
  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/cm(2) (+11.1%; ie, +2.05% per year) without recurrence of vertebral fracture.
  
  
  CONCLUSIONS
  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.
authors.affiliation: AP-HP, Dermatology and Allergy Department, Université Pierre et Marie Curie, Tenon Hospital, 4 rue Chine, Paris 75020, France. stephane.barete@psl.aphp.fr
authors.forename: S
authors.initials: S
authors.lastname: Barete
authors.forename: N
authors.initials: N
authors.lastname: Assous
authors.forename: C
authors.initials: C
authors.lastname: de Gennes
authors.forename: C
authors.initials: C
authors.lastname: Grandpeix
authors.forename: F
authors.initials: F
authors.lastname: Feger
authors.forename: F
authors.initials: F
authors.lastname: Palmerini
authors.forename: P
authors.initials: P
authors.lastname: Dubreuil
authors.forename: M
authors.initials: M
authors.lastname: Arock
authors.forename: C
authors.initials: C
authors.lastname: Roux
authors.forename: J M
authors.initials: JM
authors.lastname: Launay
authors.forename: S
authors.initials: S
authors.lastname: Fraitag
authors.forename: D
authors.initials: D
authors.lastname: Canioni
authors.forename: B
authors.initials: B
authors.lastname: Billemont
authors.forename: F
authors.initials: F
authors.lastname: Suarez
authors.forename: F
authors.initials: F
authors.lastname: Lanternier
authors.forename: O
authors.initials: O
authors.lastname: Lortholary
authors.forename: O
authors.initials: O
authors.lastname: Hermine
authors.forename: C
authors.initials: C
authors.lastname: Francès
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015519:Bone Density / Q000187:drug effects; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D004164:Diphosphonates / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008159:Lumbar Vertebrae / Q000503:physiopathology; D008297:Male; D034721:Mastocytosis, Systemic / Q000150:complications* / Q000503:physiopathology; D008875:Middle Aged; D010024:Osteoporosis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D055815:Young Adult
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates