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Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
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Focal Segmental Glomerulosclerosis Associated with Essential Thrombocythemia

Hisashi Sugimoto1, Naoki Sawa1, Hajime Yamagiwa1

  • 1Nephrology Center and the Okinaka Memorial Institute for Medical Research, Toranomon Hospital, Japan.

Internal Medicine (Tokyo, Japan)
|October 26, 2022
PubMed

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, 57.9×104/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.

Keywords:
essential thrombocythemiafocal segmental glomerulosclerosisnephrotic syndrome

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