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马法兰、地塞米松、沙利度胺方案成功治疗复发难治特发性轻链沉积肾病一例报告及文献复习 被引量:1

Successful treatment with melphalan, dexamethasone and thalidomide for relapsed idiopathic light chain deposit disease: a case report and literature review
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摘要 轻链沉积病(LCDD)是克隆性浆细胞病中的罕见类型,很多器官均可能因轻链沉积导致功能受损,例如肝、心和胃肠道等,肾脏是LCDD最常见的受累脏器.LCDD肾病最常见的临床表现包括肾功能减退、蛋白尿和肾病综合征等[1].约50%的LCDD继发于多发性骨髓瘤(MM)和淋巴细胞增殖性疾病[2],其余患者则无任何其他疾病的证据,可以考虑为特发性LCDD.由于本病需要病理确诊,而且多数都是重要脏器受累,因此,以往的漏诊和误诊率较高,近年来由于活检技术和免疫病理技术的进步,本病检出率虽有所增高,但仍然系罕见疾病,尤其是不伴有其他基础疾病的特发性LCDD.
出处 《中华血液学杂志》 CAS CSCD 北大核心 2015年第1期60-62,共3页 Chinese Journal of Hematology
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  • 1Preud' homme JL, Aucouturier P, Touchard G, et al. Monoclonal immunoglobulin deposition disease (Randall type). Relation- ship with structural abnormalities of immunoglobulin chains [ J]. Kidney Int, 1994, 46(4): 965-972.
  • 2Heilman RL, Velosa JA, Holley KE, et al. Long-term follow-up and response to chemotherapy in patients with light-chain depo- sition disease[J]. Am J Kidney Dis, 1992, 20( 1 ): 34-41.
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  • 7Okura T, Miyoshi K, Nagao T, et al. Light chain deposition disease developing 15 years following the diagnosis of monoclonal gammopathy of undetermined significance [J]. Intern Med, 2009, 48(2): 101-104.
  • 8Reiter E, Kalhs P, Keil F, et al. Effect of high-dose melphalan and peripheral blood stem cell transplantation on renal function in patients with multiple myeloma and renal insufficiency: a case report and review of the literature [J]. Ann Hematol, 1999, 78(4): 189-191.
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