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Diagnosis: Renal Crystal-Storing Histiocytosis Involving Glomeruli and Interstitium
Crystal-storing histiocytosis (CSH) is a rare manifestation of monoclonal gammopathy in which histiocytes containing monoclonal proteins in their cytoplasm are found in various organs of the body including the kidney. Within the kidney, these monoclonal crystal-laden histiocytes have been described to occur in the interstitium (most commonly) or in glomeruli. Most cases involve IgG kappa crystals. Patients with CSH predominantly involving the glomerular capillaries showed a trend to- ward lower serum creatinine and proteinuria at presentation, and several patients with CSH lacked a definitive diagnosis of a monoclonal gammopathy at the time of diagnosis, emphasizing the role that kidney biopsy in diagnosis of this entity (From: Gupta RK, Rosenberg AZ, Bagnasco SM, Arend LJ. Renal crystal-storing histiocytosis involving glomeruli - A comprehensive clinicopathologic analysis. Ann Diagn Pathol. 2019 Dec;43:151403. [PubMed link]).
See the chapter: Amyloidosis and renal involvement in monoclonal gammopathies of our Tutorial
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References
- Qin AB, Yu XJ, Zheng XZ, Wang SX, Zhou FD, Zhao MH. Lessons for the clinical nephrologist: a rare case with MGRS characterized by combined crystalline light chain proximal tubulopathy and crystal-storing histiocytosis responding to daratumumab. J Nephrol. 2023 Mar 1. Epub ahead of print. [PubMed link].
- Sy-Go JPT, Herrmann SM, Seshan SV. Monoclonal Gammopathy-Related Kidney Diseases. Adv Chronic Kidney Dis. 2022;29(2):86-102.e1. [PubMed link]
- Gupta RK, Rosenberg AZ, Bagnasco SM, Arend LJ. Renal crystal-storing histiocytosis involving glomeruli - A comprehensive clinicopathologic analysis. Ann Diagn Pathol. 2019;43:151403. [PubMed link]
- Galeano-Valle F, Díaz-Crespo FJ, Melero-Martín R, Apaza-Chávez JE, Del-Toro-Cervera J, Demelo-Rodríguez P. Massive generalized crystal-storing histiocytosis associated with extracellular crystalline nephropathy: clinical, immunohistochemical, and ultrastructural studies of a unique disorder and review of the literature. CEN Case Rep. 2019;8(3):166-172. [PubMed link
- Boudhabhay I, Titah C, Talbot A, Harel S, Verine J, Touchard G, Kaaki S, Gabison E, Vasseur V, Mauget-Faÿsse M, Sené T. Multiple myeloma with crystal-storing histiocytosis, crystalline podocytopathy, and light chain proximal tubulopathy, revealed by retinal abnormalities: A case report. Medicine (Baltimore). 2018;97(52):e13638. [PubMed link]
- Kaur A, Sethi S. Histiocytic and Nonhistiocytic Glomerular Lesions: Foam Cells and Their Mimickers. Am J Kidney Dis. 2016;67(2):329-36. [PubMed link]