High dose chemotherapy and stem cell support in a patient of light- and heavy-chain deposition disease with abnormal marrow cell surface antigens and no monoclonal protein
High dose chemotherapy and stem cell support in a patient of light- and heavy-chain deposition disease with abnormal marrow cell surface antigens and no monoclonal protein
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DOI：
10.2169/internalmedicine.44.970
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发表时间：
2005-09-01
影响因子：
1.2
通讯作者：
Hishida, A
中科院分区：
文献类型：
--
作者：
Sakakima, M;Fujigaki, Y;Hishida, A
一名患有肾病综合征和严重肾功能衰竭的 53 岁男性，经肾活检诊断为轻链和重链沉积病 (LHCDD)。该患者没有单克隆蛋白和轻度骨髓浆细胞增多症（6%），但骨髓浆细胞表达CD19（-）CD56（+）和主要的单克隆κ链，表明浆细胞恶液质。常规化疗无效且不能改善肾衰竭。即使在血液透析后也引入高剂量化疗/外周血干细胞移植（HDC/PBSCT），以消除异常克隆并使骨髓细胞表面标记物正常化。骨髓细胞的免疫表型分析有助于对无单克隆蛋白的 LHCDD 患者使用 HDC/PBSCT 进行临床决策。
A 53-year-old man with nephrotic syndrome and severe renal failure was diagnosed with light- and heavy-chain deposition disease (LHCDD) by renal biopsy. The patient had no monoclonal protein and mild marrow plasmacytosis (6%), but marrow plasma cells expressed CD19(-)CD56(+) and predominant monoclonal kappa-chain, indicating plasma cell dyscrasia. Conventional chemotherapy was ineffective and did not improve renal failure. High dose chemotherapy/peripheral blood stem cell transplantation (HDC/PBSCT) was introduced even after hemodialysis to eliminate aberrant clone and normalization of bone marrow cell surface markers. Immuno-phenotypic analysis of marrow cells facilitates clinical decision making regarding the use of HDC/PBSCT for LHCDD patients without monoclonal protein.