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轻链晶体沉积作为浆细胞异常增殖性疾病的一种表现:免疫电子显微镜的作用

Light chain crystal deposition as a manifestation of plasma cell dyscrasias: the role of immunoelectron microscopy.

作者信息

Gu Xin, Barrios Roberto, Cartwright Joiner, Font Ramon L, Truong Luan, Herrera Guillermo A

机构信息

Louisiana State University Health Sciences Center, Shreveport, LA, USA.

出版信息

Hum Pathol. 2003 Mar;34(3):270-7. doi: 10.1053/hupa.2003.27.

Abstract

Light chain crystal deposition disease is a rare and poorly characterized entity that can be confused with a number of different conditions, depending on where the disease process is manifested. The present study explored the role of ultrastructural immunogold labeling in the diagnosis of this condition. Seven cases of light chain crystal deposition (kappa light chain-related) are reported. Immunohistochemistry and immunofluorescence techniques play a rather limited role in the evaluation of these cases, as a result of the inability to detect monoclonal kappa light chains in association with the crystalline structures or high background staining. Ultrastructural labeling is the method of choice to fully characterize these cases. However, surgical pathologists must learn to recognize the findings associated with this condition to avoid misdiagnosis. If the diagnosis is at least suspected, then a complete hematologic workup may identify the underlying plasma cell dyscrasia. It must be emphasized that in some patients the plasma cell dyscrasia does not become clinically manifested until years after the diagnosis of light chain crystal deposition.

摘要

轻链晶体沉积病是一种罕见且特征不明的疾病,根据疾病过程的表现部位,它可能与多种不同病症相混淆。本研究探讨了超微结构免疫金标记在该病症诊断中的作用。报告了7例轻链晶体沉积(与κ轻链相关)病例。免疫组织化学和免疫荧光技术在评估这些病例时作用相当有限,因为无法检测到与晶体结构相关的单克隆κ轻链或存在高背景染色。超微结构标记是全面表征这些病例的首选方法。然而,外科病理学家必须学会识别与该病症相关的发现以避免误诊。如果至少怀疑有该诊断,那么完整的血液学检查可能会识别出潜在的浆细胞异常增殖症。必须强调的是,在一些患者中,浆细胞异常增殖症直到轻链晶体沉积诊断多年后才会出现临床症状。

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