关键词: fracture hip mastocytosis osteoporosis osteosclerosis

来  源:   DOI:10.1016/j.aace.2023.10.003   PDF(Pubmed)

Abstract:
UNASSIGNED: Patients with systemic mastocytosis are at high risk of developing osteoporosis and fractures. Herein, we report a case of hip fragility fracture in a patient with indolent systemic mastocytosis and normal bone density.
UNASSIGNED: A 48-year-old man experienced a left femoral neck fracture after a fall. After a dose of oxycodone/hydromorphone postoperatively, he developed an anaphylactic reaction. Previously, he experienced a few other episodes of flushing, dizziness, and syncope precipitated by stress and alcohol. His examination was notable for pink and brown macules on his chest, back, arms, and legs. His laboratory test revealed a markedly elevated tryptase level of 171 ng/mL (<11 ng/mL). Treatment including cetirizine, montelukast, and ranitidine controlled his symptoms. His bone density test result was normal. Ten months after hip surgery, his c-terminal telopeptide of collagen type 1 and bone-specific alkaline phosphatase levels significantly increased. The bone scan demonstrated diffusely increased radiotracer uptake throughout the osseous structures. Given high bone turnover and the prior hip fracture, he received zoledronic acid yearly for 3 years, and no further fractures have occurred.
UNASSIGNED: The case is unusual as the fracture occurred despite normal bone density and significant osteosclerosis, which was previously considered protective against fractures. Additionally, rather than the spine, the fracture occurred in the hip, which is an uncommon site for mastocytosis-induced fractures.
UNASSIGNED: Mastocytosis is a rare cause of osteoporosis, and it is important to keep this condition in the differential diagnosis of osteoporosis, particularly when the fracture presentation is atypical.
摘要:
全身性肥大细胞增多症患者发生骨质疏松和骨折的风险很高。在这里,我们报告了一例伴有系统性肥大细胞增多症和骨密度正常的髋部脆性骨折。
一名48岁男子跌倒后出现左侧股骨颈骨折。术后一定剂量的羟考酮/氢吗啡酮,他出现了过敏反应。以前,他还经历了几次潮红,头晕,压力和酒精引起的晕厥。他的检查值得注意的是他的胸部有粉红色和棕色的斑点,回来,武器,和腿。他的实验室测试显示,类胰蛋白酶水平显着升高,为171ng/mL(<11ng/mL)。治疗包括西替利嗪,孟鲁司特,雷尼替丁控制了他的症状.他的骨密度测试结果正常。髋关节手术后十个月,他的1型胶原蛋白的c末端端肽和骨特异性碱性磷酸酶水平显着增加。骨扫描显示整个骨结构的放射性示踪剂摄取增加。鉴于高的骨转换和先前的髋部骨折,他每年接受唑来膦酸治疗3年,没有再发生骨折。
这种情况是不寻常的,因为尽管骨密度正常且骨硬化明显,但仍发生了骨折,以前被认为对骨折有保护作用。此外,而不是脊柱,骨折发生在髋部,这是肥大细胞增多引起的骨折的罕见部位。
肥大细胞增多是一种罕见的骨质疏松病因,在骨质疏松症的鉴别诊断中保持这种情况很重要,特别是当骨折表现不典型时。
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