Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review
Background Crystal-storing histiocytosis (CSH), light chain proximal tubulopathy (LCPT), and light chain crystalline podocytopathy (LCCP) are rare complications of multiple myeloma (MM) or monoclonal gammopathy of renal significance, and their diagnoses are challenging.Case presentation In this case...
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| Format: | Article |
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Taylor & Francis Group
2023-12-01
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| Series: | Renal Failure |
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| Online Access: | https://www.tandfonline.com/doi/10.1080/0886022X.2022.2145970 |
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| author | Li Zhu Lei Wang Hongxia Shi Lei Jiang Xin Li Chunying Shao Yu Yan Bao Dong Wanzhong Zou Li Zuo |
| author_facet | Li Zhu Lei Wang Hongxia Shi Lei Jiang Xin Li Chunying Shao Yu Yan Bao Dong Wanzhong Zou Li Zuo |
| author_sort | Li Zhu |
| collection | DOAJ |
| description | Background Crystal-storing histiocytosis (CSH), light chain proximal tubulopathy (LCPT), and light chain crystalline podocytopathy (LCCP) are rare complications of multiple myeloma (MM) or monoclonal gammopathy of renal significance, and their diagnoses are challenging.Case presentation In this case, a 69-year-old Chinese woman presented with suspicious Fanconi syndrome with renal insufficiency. Immunofixation electrophoresis of both serum and urine revealed elevated immunoglobulin G kappa (IgGkappa) and kappa light chain. Bone marrow aspirate revealed 15% plasma cells with considerable cytoplasmic granular inclusions and needle-shaped crystals. Renal biopsy confirmed the final pathologic diagnosis of kappa-restricted CSH, combined LCPT and LCCP by immunoelectron microscopy. A number of special casts were present which could easily be misdiagnosed as light chain cast nephropathy. Immunofluorescence on frozen tissue presented false negative for kappa light chain, as ultimately proven by paraffin-embedded tissue after pronase digestion. MM and CSH were diagnosed, and two cycles of chemotherapy were given. The patient subsequently refused further chemotherapy, and her renal function remained relatively stable during a 2.5-year follow-up period.Conclusions In conclusion, we report a rare case of generalized kappa-restricted CSH involving bone marrow and kidney, combined with LCPT and LCCP, provide a comprehensive summary of renal CSH, and propose a new nomenclature of monoclonal immunoglobulin-induced crystalline nephrology. The presentation of monoclonal immunoglobulin and Fanconi syndrome should suggest the presence of monoclonal immunoglobulin-induced crystalline nephrology. Use of paraffin-embedded tissue after pronase digestion and immunoelectron microscopy is beneficial to improve the sensitivity of diagnosis. |
| format | Article |
| id | doaj-art-ca463e81fa2a4cbeb836507ee6b73c12 |
| institution | DOAJ |
| issn | 0886-022X 1525-6049 |
| language | English |
| publishDate | 2023-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Renal Failure |
| spelling | doaj-art-ca463e81fa2a4cbeb836507ee6b73c122025-08-20T02:56:11ZengTaylor & Francis GroupRenal Failure0886-022X1525-60492023-12-0145110.1080/0886022X.2022.2145970Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature reviewLi Zhu0Lei Wang1Hongxia Shi2Lei Jiang3Xin Li4Chunying Shao5Yu Yan6Bao Dong7Wanzhong Zou8Li Zuo9Department of Nephrology, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaElectron Microscope Lab, Peking University People’s Hospital, Beijing, ChinaElectron Microscope Lab, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaDepartment of Nephrology, Peking University People’s Hospital, Beijing, ChinaBackground Crystal-storing histiocytosis (CSH), light chain proximal tubulopathy (LCPT), and light chain crystalline podocytopathy (LCCP) are rare complications of multiple myeloma (MM) or monoclonal gammopathy of renal significance, and their diagnoses are challenging.Case presentation In this case, a 69-year-old Chinese woman presented with suspicious Fanconi syndrome with renal insufficiency. Immunofixation electrophoresis of both serum and urine revealed elevated immunoglobulin G kappa (IgGkappa) and kappa light chain. Bone marrow aspirate revealed 15% plasma cells with considerable cytoplasmic granular inclusions and needle-shaped crystals. Renal biopsy confirmed the final pathologic diagnosis of kappa-restricted CSH, combined LCPT and LCCP by immunoelectron microscopy. A number of special casts were present which could easily be misdiagnosed as light chain cast nephropathy. Immunofluorescence on frozen tissue presented false negative for kappa light chain, as ultimately proven by paraffin-embedded tissue after pronase digestion. MM and CSH were diagnosed, and two cycles of chemotherapy were given. The patient subsequently refused further chemotherapy, and her renal function remained relatively stable during a 2.5-year follow-up period.Conclusions In conclusion, we report a rare case of generalized kappa-restricted CSH involving bone marrow and kidney, combined with LCPT and LCCP, provide a comprehensive summary of renal CSH, and propose a new nomenclature of monoclonal immunoglobulin-induced crystalline nephrology. The presentation of monoclonal immunoglobulin and Fanconi syndrome should suggest the presence of monoclonal immunoglobulin-induced crystalline nephrology. Use of paraffin-embedded tissue after pronase digestion and immunoelectron microscopy is beneficial to improve the sensitivity of diagnosis.https://www.tandfonline.com/doi/10.1080/0886022X.2022.2145970Crystal-storing histiocytosismultiple myelomalight chain proximal tubulopathylight chain crystalline podocytopathymonoclonal immunoglobulin-induced crystalline nephrology |
| spellingShingle | Li Zhu Lei Wang Hongxia Shi Lei Jiang Xin Li Chunying Shao Yu Yan Bao Dong Wanzhong Zou Li Zuo Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review Renal Failure Crystal-storing histiocytosis multiple myeloma light chain proximal tubulopathy light chain crystalline podocytopathy monoclonal immunoglobulin-induced crystalline nephrology |
| title | Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review |
| title_full | Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review |
| title_fullStr | Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review |
| title_full_unstemmed | Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review |
| title_short | Combined crystal-storing histiocytosis, light chain proximal tubulopathy, and light chain crystalline podocytopathy in a patient with multiple myeloma: a case report and literature review |
| title_sort | combined crystal storing histiocytosis light chain proximal tubulopathy and light chain crystalline podocytopathy in a patient with multiple myeloma a case report and literature review |
| topic | Crystal-storing histiocytosis multiple myeloma light chain proximal tubulopathy light chain crystalline podocytopathy monoclonal immunoglobulin-induced crystalline nephrology |
| url | https://www.tandfonline.com/doi/10.1080/0886022X.2022.2145970 |
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