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Clinicopathological characteristics of light chain proximal tubulopathy in Korean patients and the diagnostic usefulness of immunohistochemical staining for immunoglobulin light chain
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Jung, Minsun | - |
dc.contributor.author | Lee, Youngeun | - |
dc.contributor.author | Lee, Hajeong | - |
dc.contributor.author | Moon, Kyung Chul | - |
dc.date.accessioned | 2020-08-11T09:02:17Z | - |
dc.date.available | 2020-08-11T09:02:17Z | - |
dc.date.issued | 2020-04-23 | - |
dc.identifier.citation | BMC Nephrology, 21(1):146 | ko_KR |
dc.identifier.issn | 1471-2369 | - |
dc.identifier.uri | https://hdl.handle.net/10371/168709 | - |
dc.description.abstract | Light chain proximal tubulopathy (LCPT) is a rare paraproteinemic renal disease that has been mostly reported in Western patients. LCPT is characterized by the accumulation of immunoglobulin (Ig)-light chain (LC) in the proximal tubule. Immunohistochemical staining for Ig-LC has not been investigated in the context of LCPT. We reported the clinicopathological characteristics and Ig-LC immunoexpression of patients with LCPT for the first time in Korea.
We reviewed the clinicopathological findings of 5 Korean patients diagnosed with LCPT between 2016 and 2018. In addition, immunohistochemical staining for κ-LC and λ-LC was conducted on paraffin-embedded tissues. The median age was 63 years, and the male-to-female ratio was 3:2. The primary renal manifestations were either azotemia or tubular proteinuria. All patients were diagnosed with multiple myeloma with monoclonal κ-LC (#1–2) or λ-LC (#3–5) in the serum and urine. Kidney biopsies revealed diverse and subtle alterations of the proximal tubule, including crystallization, vacuolization, and/or swelling. Electron microscopy revealed crystals in patients #1–2 and non-crystalline particles within numerous/large/dysmorphic lysosomes in patients #3–5. Ig-LC restriction was demonstrated in the proximal tubule as κ-type in patients #1–2 and as λ-type in patients #3–5 by immunohistochemistry and immunofluorescence. Immunohistochemical staining showed diffuse positivity to κ- and λ-LC, although immunofluorescent staining for κ-LC was focal and weak. LCPT has diverse clinicopathological characteristics and subtle morphological alterations, which necessitate ancillary tests for diagnosis. We introduced immunohistochemical staining for Ig-LC as a useful tool for the diagnosis of LCPT, especially in the case of κ-type crystals. | ko_KR |
dc.language.iso | en | ko_KR |
dc.publisher | BMC | ko_KR |
dc.subject | Immunoglobulin light chains | - |
dc.subject | Immunohistochemistry | - |
dc.subject | Light chain proximal Tubulopathy | - |
dc.subject | Monoclonal Gammopathy | - |
dc.subject | Multiple myeloma | - |
dc.title | Clinicopathological characteristics of light chain proximal tubulopathy in Korean patients and the diagnostic usefulness of immunohistochemical staining for immunoglobulin light chain | ko_KR |
dc.type | Article | ko_KR |
dc.contributor.AlternativeAuthor | 정민선 | - |
dc.contributor.AlternativeAuthor | 이영은 | - |
dc.contributor.AlternativeAuthor | 이하정 | - |
dc.contributor.AlternativeAuthor | 문경철 | - |
dc.identifier.doi | 10.1186/s12882-020-01813-w | - |
dc.language.rfc3066 | en | - |
dc.rights.holder | The Author(s) | - |
dc.date.updated | 2020-06-17T13:04:00Z | - |
dc.citation.endpage | 151 | ko_KR |
dc.citation.number | 1 | ko_KR |
dc.citation.startpage | 146 | ko_KR |
dc.citation.volume | 21 | ko_KR |
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