Nephrology Center and the Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan.
Department of Hematology, Toranomon Hospital, Japan.
Intern Med. 2023 Jun 15;62(12):1789-1794. doi: 10.2169/internalmedicine.0767-22. Epub 2022 Oct 26.
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.
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。