国际医药卫生导报2024,Vol.30Issue(9) :1552-1555.DOI:10.3760/cma.j.issn.1007-1245.2024.09.032

肾轻链沉积病伴多浆膜腔积液1例

A case of renal light chain deposition disease complicated with multiple plasma chamber effusion

徐田田
国际医药卫生导报2024,Vol.30Issue(9) :1552-1555.DOI:10.3760/cma.j.issn.1007-1245.2024.09.032

肾轻链沉积病伴多浆膜腔积液1例

A case of renal light chain deposition disease complicated with multiple plasma chamber effusion

徐田田1
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作者信息

  • 1. 暨南大学附属第一医院血液内科,广州 510630
  • 折叠

摘要

轻链沉积病(LCDD)是最多见的单克隆免疫球蛋白沉积病,由浆细胞或B淋巴细胞分泌过量单克隆免疫球蛋白轻链,沉积在组织中引起器官功能障碍.LCDD多表现为以肾脏为首的多器官受累全身性疾病.肾脏受累几乎涉及所有LCDD患者,肾外受累则相对较少,其中心脏、肝脏、胃肠道、周围神经和自主神经受累较为常见,肺部受累非常罕见.LCDD脏器功能受损多数呈进行性发展,罕有自发缓解.肾外轻链沉积对患者生存率及预后有明确的独立影响.

Abstract

Light chain deposition disease(LCDD)is the most common form of monoclonal immunoglobulin deposition disease,in which plasma cells or B-lymphocytes secrete excessive amounts of monoclonal immunoglobulin light chains,which are deposited in tissues and cause organ dysfunction.LCDD is a systemic disease with multiple organ involvement,mainly in the kidney.Renal involvement is common in almost all patients with LCDD,while extra-renal involvement is less common,with cardiac,hepatic,gastrointestinal,peripheral,and autonomic nerve involvement being more common,and pulmonary involvement being very rare.Most of the organ impairment in LCDD is progressive,and spontaneous remission is rare.Extra-renal light chain deposition has clear independent effect on the patients'survival and prognosis.

关键词

肾功能衰竭/肾轻链沉积病/多浆膜腔积液/硼替佐米/诊断及治疗

Key words

Renal failure/Renal light chain deposition disease/Multiple plasma lumen effusion/Bortezomib/Diagnosis and treatment

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出版年

2024
国际医药卫生导报
中华医学会,国际医药卫生导报社

国际医药卫生导报

影响因子:0.781
ISSN:1007-1245
参考文献量32
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