BACKGROUND: The mechanism of osteoporosis with fracture secondary to mastocytosis is little known, and its treatment is poorly codified. METHODS: Ten patients with a mean age of 52.5 years with systemic mastocytosis and osteoporotic fractures were treated with interferon alpha 1.5 million U 3 times per week, combined with monthly pamidronate infusions (1 mg/kg) for 2 years, followed by pamidronate infusions every 3 months. RESULTS: Before treatment, the mean number of vertebral fractures was 3.5, spinal T-score was -3±1, hip T-score was -1.9±0.7, serum C-terminal telopeptide was 357±258 pg/mL (N=80-800), bone alkaline phosphatase was 20±3.2 IU (N=8-25), and tryptase was 49±36 μg/mL (N<10). Interferon alpha was discontinued in 2 patients because of poor tolerance. Mean follow-up was 60 months. No patient developed a fracture under treatment. In the 8 patients treated with interferon alpha and pamidronate, the mean annual increase in spinal bone mineral density was 12.6%±5.6% and 1.93% in hip bone mineral density. Serum C-terminal telopeptide decreased by 66%, bone alkaline phosphatase decreased by 25%, and tryptase decreased by 34%. In the 2 patients treated with pamidronate alone, mean annual bone mineral density increase was 2.4%±0.1% at the spine and 0%±01% at the hip. CONCLUSION: Osteoporosis secondary to mastocytosis mainly affects trabecular bone, and markers of bone remodeling are normal. Combined treatment with low doses of interferon and pamidronate markedly increased bone density.
BACKGROUND: The mechanism of osteoporosis with fracture secondary to mastocytosis is little known, and its treatment is poorly codified. METHODS: Ten patients with a mean age of 52.5 years with systemic mastocytosis and osteoporotic fractures were treated with interferon alpha 1.5 million U 3 times per week, combined with monthly pamidronate infusions (1 mg/kg) for 2 years, followed by pamidronate infusions every 3 months. RESULTS: Before treatment, the mean number of vertebral fractures was 3.5, spinal T-score was -3±1, hip T-score was -1.9±0.7, serum C-terminal telopeptide was 357±258 pg/mL (N=80-800), bone alkaline phosphatase was 20±3.2 IU (N=8-25), and tryptase was 49±36 μg/mL (N<10). Interferon alpha was discontinued in 2 patients because of poor tolerance. Mean follow-up was 60 months. No patient developed a fracture under treatment. In the 8 patients treated with interferon alpha and pamidronate, the mean annual increase in spinal bone mineral density was 12.6%±5.6% and 1.93% in hip bone mineral density. Serum C-terminal telopeptide decreased by 66%, bone alkaline phosphatase decreased by 25%, and tryptase decreased by 34%. In the 2 patients treated with pamidronate alone, mean annual bone mineral density increase was 2.4%±0.1% at the spine and 0%±01% at the hip. CONCLUSION:Osteoporosis secondary to mastocytosis mainly affects trabecular bone, and markers of bone remodeling are normal. Combined treatment with low doses of interferon and pamidronate markedly increased bone density.
Authors: S Ferrari; M L Bianchi; J A Eisman; A J Foldes; S Adami; D A Wahl; J J Stepan; M-C de Vernejoul; J-M Kaufman Journal: Osteoporos Int Date: 2012-06-09 Impact factor: 4.507
Authors: M Rossini; R Zanotti; G Orsolini; G Tripi; O Viapiana; L Idolazzi; A Zamò; P Bonadonna; V Kunnathully; S Adami; D Gatti Journal: Osteoporos Int Date: 2016-02-18 Impact factor: 4.507
Authors: Gerhard J Molderings; Britta Haenisch; Stefan Brettner; Jürgen Homann; Markus Menzen; Franz Ludwig Dumoulin; Jens Panse; Joseph Butterfield; Lawrence B Afrin Journal: Naunyn Schmiedebergs Arch Pharmacol Date: 2016-04-30 Impact factor: 3.000