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特发性血小板增多症相关局灶节段性肾小球硬化症。

Focal Segmental Glomerulosclerosis Associated with Essential Thrombocythemia.

机构信息

Nephrology Center and the Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan.

Department of Hematology, Toranomon Hospital, Japan.

出版信息

Intern Med. 2023 Jun 15;62(12):1789-1794. doi: 10.2169/internalmedicine.0767-22. Epub 2022 Oct 26.

Abstract

A 72-year-old man was admitted for examination of proteinuria (9.14 g/day) and leg edema. Essential thrombocythemia (ET) was diagnosed because of thrombocytosis (platelet count, 57.9×10/μL), elevated megakaryocytes in bone marrow biopsy, and JAK2 V617 mutation. Kidney biopsy led to a diagnosis of focal segmental glomerulosclerosis (FSGS) cellular variant (characterized by glomerular capillaries filled with swollen endothelial cells containing foam cells) in 6 glomeruli, FSGS tip variant in 5 glomeruli, and additional FSGS variants in other glomeruli. Affected glomeruli had anti-CD61 antibody staining-positive megakaryocyte infiltrations. ET mayinduce FSGS because megakaryocyte infiltration increases intraglomerular pressure, resulting in hypertension and proteinuria.

摘要

一位 72 岁男性因蛋白尿(9.14g/天)和腿部水肿入院检查。由于血小板增多症(血小板计数 57.9×10/μL)、骨髓活检中巨核细胞升高,以及 JAK2 V617 突变,诊断为特发性血小板增多症(ET)。肾活检在 6 个肾小球中诊断为局灶节段性肾小球硬化症(FSGS)细胞型(特征为充满肿胀内皮细胞的肾小球毛细血管,其中含有泡沫细胞),在 5 个肾小球中诊断为 FSGS 尖端型,在其他肾小球中诊断为其他 FSGS 变异型。受影响的肾小球有抗 CD61 抗体染色阳性的巨核细胞浸润。ET 可能会引起 FSGS,因为巨核细胞浸润会增加肾小球内压,导致高血压和蛋白尿。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/431a/10332981/7de4d08edf2c/1349-7235-62-1789-g001.jpg

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