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医学博文41-一例报结节性多动脉炎合并多发性骨髓瘤·····

标签:多发性骨髓瘤 医学科普 | 作者:张莉芸 | 发表时间:2014-02-08 16:32:35

一例报结节性多动脉炎合并多发性骨髓瘤报道和相关文献复习

译者:刘樱 校对者:庞宇洁 审核者:张莉芸

Polyarteritis nodosa complicating multiple myeloma - a case report and review of the literature

Introduction: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of smallto-medium-sized vessels, rarely associated with hematologic neoplasms. Case report: We report a 44-year-old man with a history of monoclonal gammopathy of undetermined significance (MGUS) who presented with rapidly progressing sensorimotor peripheral neuropathy. Two weeks after onset the patient developed severe acute acral and retinal ischemia. MR-angiography and nerve biopsy revealed a systemic necrotizing vasculitis (PAN type). At this time, bone marrow biopsy identified a smoldering multiple myeloma. Immediate immunosuppressive and anti-neoplastic treatment (steroids, immunoglobulins, bortezomib combined with cyclophosphamide followed by lenalidomide maintenance) resulted in a favorable clinical outcome. After 4 years, the patient is in good clinical condition with sustained partial remission from myeloma and without evidence of relapse of PAN. Conclusion: This is a remarkable case of a histologically confirmed peripheral neuropathy due to polyarteritis nodosa associated with progression of MGUS to multiple myeloma. immediate diagnosis and combined immunosuppressive and anti-neoplastic treatment may improve the outcome of this potentially life-threatening clinical condition.

· 结节性多动脉炎是关于小中型血管的坏死性血管炎,但很少会产生血液肿瘤。简述:患者,44岁,有意义未明单克隆免疫球蛋白血症史,临床表现为快速进展的外周运动感觉神经病变, 2星期后开始出现严重急性肢端和视网膜缺血。血管造影术和神经活检发现为结节性多动脉炎。与此同时,骨髓组织活检证实患者有多发性骨髓瘤。立即给予免疫抑制剂和抗肿瘤治疗(激素、硼替佐米、环磷酰胺随后使用来那度胺维持治疗)后出现较好的临床缓解。在以后的4年里,患者身体状况良好,骨髓瘤得到长期部分缓解,并且结节性多动脉炎并没有复发的证据。结论:患者明确诊断为结节性多动脉炎、未明单克隆免疫球蛋白血症、多发性骨髓瘤、外周神经病变。快速诊断、联合免疫抑制剂和抗肿瘤治疗也许会提高这种潜在的危急生命的临床症状的治疗效果。


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