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ArticleNephron2026

Lysozyme-Associated Nephropathy in Myeloid Neoplasms: A Clinicopathological and Mass Spectrometric Study of Two Cases.

Minami Matsumoto, Shinya Yamamoto, Kanna Shinkawa, Takeshi Matsubara, June Takeda, Toshio Kitawaki, Yasuyuki Arai, Yuki Teramoto, Sachiko Minamiguchi, Yukiko Kanetsuna and 8 more

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Nephron, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

18 authors.

Minami MatsumotoDepartment of Nephrology, Kyoto University, Kyoto, Japan.
Shinya YamamotoDepartment of Nephrology, Kyoto University, Kyoto, Japan, syamamon@kuhp.kyoto-u.ac.jp.
Kanna ShinkawaDepartment of Nephrology, Kyoto University, Kyoto, Japan.
Takeshi MatsubaraDepartment of Nephrology, Kyoto University, Kyoto, Japan.
June TakedaDepartment of Hematology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Toshio KitawakiDepartment of Hematology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Yasuyuki AraiDepartment of Hematology, Graduate School of Medicine, Kyoto University, Kyoto, Japan.
Yuki TeramotoDepartment of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
Sachiko MinamiguchiDepartment of Diagnostic Pathology, Kyoto University Hospital, Kyoto, Japan.
Yukiko KanetsunaDepartment of Pathology, International University of Health and Welfare School of Medicine Atami Hospital, Shizuoka, Japan.
Kensuke JohDepartment of Pathology, The Jikei University School of Medicine, Tokyo, Japan.
Yutaka YamaguchiYamaguchi's Pathology Laboratory, Chiba, Japan.
Dedong KangDepartment of Anatomy, Showa Medical University School of Medicine, Tokyo, Japan.
Kazuho HondaDepartment of Anatomy, Showa Medical University School of Medicine, Tokyo, Japan.
Akiko MiiDepartment of Endocrinology, Metabolism and Nephrology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan.
Akira ShimizuDepartment of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan.
Yoshifumi UbaraNephrology Center, Toranomon Hospital Kajigaya, Kawasaki, Japan.
Motoko YanagitaDepartment of Nephrology, Kyoto University, Kyoto, Japan.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionLysozyme-associated nephropathy (LyN) is an underrecognized cause of kidney dysfunction, commonly associated with chronic myelomonocytic leukemia (CMML). CASE PRESENTATION: We present 2 cases of LyN with distinct clinical manifestations. In Case 1, an 80-year-old man with primary myelofibrosis developed progressive monocytosis, kidney dysfunction, proteinuria, and elevated serum and urinary lysozyme levels. Kidney biopsy revealed lysozyme-positive intracytoplasmic granules within proximal tubular cells, confirming the diagnosis of LyN. Notably, the progressive increase in the number of lysozyme-positive bone marrow cells may serve as an indicator of LyN activity. Treatment with hydroxycarbamide, a cytoreductive agent, and ruxolitinib, a Janus kinase inhibitor, successfully stabilized kidney function. In Case 2, a 63-year-old man with CMML developed acute kidney injury. Although the etiology of kidney dysfunction could not be determined during life, autopsy revealed diagnostic features of LyN. To further investigate LyN pathophysiology, we performed the first mass spectrometry-based proteomic analysis of proximal tubules in 2 cases. LyN samples showed marked lysozyme accumulation and upregulation of proteins involved in lysosomal system, lipid metabolism, cellular stress, and chronic inflammation. These findings suggest that chronic lysozyme overload disrupts intracellular homeostasis and contributes to kidney injury.

conclusionLyN is underdiagnosed as unexplained tubular damage or chronic kidney disease, highlighting the need for awareness of the clinicopathological features.

Indexed as

Chronic kidney diseaseFanconi syndromeLysosomeLysozyme-associated nephropathyMass spectrometryMyeloid neoplasms

Identifiers

PMID42301980
PMCPMC13436922

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