Combined renal proximal tubulopathy and crystal storing histiocytosis in a patient with κ light chain multiple myeloma

Ungari, Marco; Ghiringhelli, Paolo; Marchi, Gianluca; Fisogni, Simona; Llombart‐Bosch, Antonio; Molteni, Alfredo; Malberti, Fabio; Bertoni, Ramona; Trombatore, Monica; Ferrero, Giuseppina; Gusolfino, Marino Daniel; Varotti, Elena; Tanzi, Giulia; Manotti, Laura

doi:10.32074/1591-951x-154

Cited by 4 publications

(2 citation statements)

References 33 publications

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“…When light chain crystal deposition is suspected, paraffin-embedded tissue should be digested with protease for antigenic repair and conformation change to better expose tissue antigenic clusters, to improve sensitivity and avoid missed diagnosis. This is consistent with the literature review [ 9 , 29 , 34 ].…”

Section: Discussionsupporting

confidence: 93%

“…To date, only 28 cases of CSH with renal involvement have been reported in the literature [ 4–27 ], some of which presented with other crystalline deposits, such as light chain proximal tubulopathy (LCPT) and light chain crystalline podocytopathy (LCCP). It is difficult to recognize and diagnose this condition because of the very low incidence and the high false-negative rate of crystals in immunofluorescence on frozen tissues [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

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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

et al. 2023

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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.

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Section: Discussionsupporting

confidence: 93%

Section: Introductionmentioning

confidence: 99%