Cundy T, Beneton M N, Darby A J, Marshall W J, Kanis J A
Bone. 1987;8(3):149-55. doi: 10.1016/8756-3282(87)90014-7.
Studies of a 63-year-old woman with osteopenia due to systemic mastocytosis are reported. Delay in making the diagnosis and the occurrence of coincident medical problems meant that radiographs during a 12-year period were available for study. Skeletal symptoms began 20 years after the skin lesions of mastocytosis first appeared, but once established bone loss was rapid, particularly from cortical bone (estimated at 3.75% pa). Biochemical and histological measurements indicated a "high turnover" bone disease and treatment with inhibitors of bone resorption was assessed. Oral, but not intravenous, clodronate was effective in reducing bone turnover as judged by falls in hydroxyproline excretion (p less than 0.01) and serum alkaline phosphatase (p less than 0.01). Mithramycin and chlorambucil were ineffective. Clodronate may be beneficial in arresting bone loss in this disorder.
本文报道了一名63岁因系统性肥大细胞增多症导致骨质减少的女性患者的研究情况。诊断延迟以及同时出现的其他医疗问题，使得我们能够获取该患者12年间的X光片进行研究。骨骼症状在肥大细胞增多症的皮肤病变首次出现20年后开始出现，但骨质流失一旦发生便迅速发展，尤其是皮质骨（估计每年流失3.75%）。生化和组织学测量结果表明这是一种“高转换型”骨病，并对使用骨吸收抑制剂进行治疗进行了评估。通过羟脯氨酸排泄量下降（p<0.01）和血清碱性磷酸酶下降（p<0.01）判断，口服氯膦酸盐（而非静脉注射）能有效降低骨转换。光辉霉素和苯丁酸氮芥无效。氯膦酸盐可能有助于阻止该疾病中的骨质流失。
