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多发性骨髓瘤伴 paget 病,颅骨病变异常伴颅内压升高。

Multiple myeloma and paget disease with abnormal skull lesions and intracranial hypertension.

机构信息

Hematology Department, S. Eugenio Hospital - Rome - Italy.

出版信息

Mediterr J Hematol Infect Dis. 2012;4(1):e2012068. doi: 10.4084/MJHID.2012.068. Epub 2012 Nov 5.

Abstract

We report a 73 years old man with a diagnosis of Paget Disease (PD) and symptomatic Multiple Myeloma (MM). Coexistence of MM and PD has rarely been described. PD mimics many of the features of bone destructive process in MM, making differential diagnosis more complicated. In addition, the presence of serious muscolo-skeletal and metabolic complications in both diseases makes management of patients difficult, worsening the prognosis.The comparison of these two diseases has led to the characterization of a common molecular mechanism represented by the receptor activator of nuclear factor-kB ligand (RANKL)/Osteoprotegerin signaling pathway. The improved comprehension of these mechanisms led to the development of new pharmacologic agents (bisphosphonates, cytokines inhibitors) effective for the treatment of these bone diseases.

摘要

我们报告了一例 73 岁男性患者,患有 Pagets 病 (PD) 和症状性多发性骨髓瘤 (MM)。MM 和 PD 同时存在的情况很少见。PD 模仿了 MM 中许多骨破坏过程的特征,使鉴别诊断更加复杂。此外,这两种疾病都存在严重的肌肉骨骼和代谢并发症,使得患者的管理变得困难,从而恶化了预后。对这两种疾病的比较导致了一种共同的分子机制的特征化,该机制由核因子-kB 配体 (RANKL)/骨保护素信号通路代表。对这些机制的深入了解导致了新的药物制剂 (双磷酸盐、细胞因子抑制剂) 的开发,这些药物制剂对这些骨骼疾病的治疗有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/21ef/3507526/1a54fa25e65a/mjhid-4-1-e2012068f1.jpg

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