Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy.
| Autor: | Kudose S; Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA., Cossey LN; Arkana Laboratories, Little Rock, Arkansas, USA., Canetta PA; Department of Medicine, Division of Nephrology, Columbia University Irving Medical Center, New York, New York, USA., Sekulic M; Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA., Vanbeek CA; AmeriPath, Oklahoma City, Oklahoma, USA., Huls FB; Department of Pathology, Division of Laboratory Medicine, University of Alabama, Birmingham, Alabama, USA., Gupta I; Middletown Medical, Middletown, New York, USA., Bu L; Mayo Clinic, Rochester, Minnesota, USA., Alexander MP; Mayo Clinic, Rochester, Minnesota, USA., Cornell LD; Mayo Clinic, Rochester, Minnesota, USA., Fidler ME; Mayo Clinic, Rochester, Minnesota, USA., Markowitz GS; Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA., Larsen CP; Arkana Laboratories, Little Rock, Arkansas, USA., D'Agati VD; Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA., Nasr SH; Mayo Clinic, Rochester, Minnesota, USA., Santoriello D; Department of Pathology and Cell Biology, Columbia University Irving Medical Center, New York, New York, USA. |
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| Jazyk: | angličtina |
| Zdroj: | Kidney international reports [Kidney Int Rep] 2023 May 15; Vol. 8 (8), pp. 1585-1595. Date of Electronic Publication: 2023 May 15 (Print Publication: 2023). |
| DOI: | 10.1016/j.ekir.2023.05.007 |
| Abstrakt: | Introduction: Lysozyme-associated nephropathy (LyN), a rare cause of kidney injury in patients with chronic myelomonocytic leukemia (CMML), has not been well described to date. We report the clinicopathologic spectrum of LyN from a multi-institutional series. Method: We identified 37 native kidney biopsies with LyN and retrospectively obtained clinicopathologic data. Results: Thirty-seven patients had a median age of 74 years and included 78% males. Their most common presentation was acute kidney injury (AKI) or AKI on chronic kidney disease (CKD) (66%) with median estimated glomerular filtration rate (eGFR) of 21.7 ml/min per 1.73 m 2 , and proteinuria of 1.7 g. A minority (15%) had partial Fanconi syndrome. Serum lysozyme levels were elevated in all tested. Hematologic disorder ( n = 28, 76%) was the most common etiology, including CMML ( n = 15), acute myeloid leukemia ( n = 5), and myelodysplastic syndrome (MDS) ( n = 5). Nonhematologic causes ( n = 5, 14%), included metastatic neuroendocrine carcinoma ( n = 3), sarcoidosis, and leprosy. Etiology was unknown in 4 (11%). Pathology showed proximal tubulopathy with abundant hypereosinophilic intracytoplasmic inclusions, with characteristic staining pattern by lysozyme immunostain. Mortality was high (8/30). However, among the 22 alive, including 85% treated, 7 had improved kidney function, including 1 who discontinued dialysis and 6 with increase in eGFR >15 ml/min per 1.73 m 2 compared with eGFR at the time of biopsy. Conclusion: Increased awareness of the full clinicopathologic spectrum of LyN may lead to prompt diagnosis, earlier treatment, and potentially improved outcome of this rare entity. (© 2023 International Society of Nephrology. Published by Elsevier Inc.) |
| Databáze: | MEDLINE |
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