Pathological findings of progressive renal involvement in a patient with TAFRO syndrome

• Published in: CEN case reports 2019-11, Vol.8 (4), p.239-245
• Main Authors: Saito, Hirotaka, Tanaka, Kenichi, Fujiwara, Momoko, Iwasaki, Tsuyoshi, Numata, Tokutaro, Oda, Akira, Kanno, Makoto, Tanaka, Mizuko, Eiro, Masaaki, Satoh, Mamoru, Kazama, Junichiro James
• Format: Article
• Language: English
• Subjects: Abdominal Pain - diagnosis; Abdominal Pain - etiology; Anti-Inflammatory Agents - administration & dosage; Anti-Inflammatory Agents - therapeutic use; Case Report; Castleman Disease - blood; Castleman Disease - diagnosis; Castleman Disease - drug therapy; Castleman Disease - pathology; Cyclosporine - administration & dosage; Cyclosporine - therapeutic use; Female; Fever - diagnosis; Fever - etiology; Glomerulonephritis, Membranoproliferative - pathology; Humans; Immunosuppressive Agents - administration & dosage; Immunosuppressive Agents - therapeutic use; Kidney - blood supply; Kidney - pathology; Kidney Glomerulus - pathology; Medicine; Medicine & Public Health; Methylprednisolone - administration & dosage; Methylprednisolone - therapeutic use; Middle Aged; Nephrology; Thrombocytopenia - diagnosis; Thrombocytopenia - etiology; Treatment Outcome; Urology; Vascular Endothelial Growth Factor A - metabolism
• ISSN: 2192-4449; 2192-4449
• DOI: 10.1007/s13730-019-00400-9

TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) is thought of as an atypical type of idiopathic multicentric Castleman’s disease. Interleukin-6, vascular endothelial growth factor (VEGF), and other cytokines are considered etiological factors. A 45-year-old woman was admitted to hospital with unknown fever and abdominal pain. She had thrombocytopenia, anasarca, proteinuria/hematuria, and slight hepatosplenomegaly. Based on her clinical course and laboratory data, she was diagnosed as having TAFRO syndrome. Kidney biopsy showed a membranoproliferative glomerulonephritis (MPGN)-like lesion containing lobulations of glomeruli, endothelial cell swelling, double contours of the glomerular basement membrane, and mesangiolysis. She was treated with methylprednisolone pulse (500 mg/day) and oral prednisolone (60 mg/day) therapy. The pleural effusion and ascites disappeared, and renal function normalized. Cyclosporine was added to prevent relapse. She went home, with no relapse 8 months after hospitalization. MPGN-like lesions were found frequently in patients with TAFRO syndrome in recent reports. However, there are few reports of pathologically confirmed cases of progressive renal involvement in TAFRO syndrome. The relationship between VEGF expression in renal tissue and the pathogenesis of renal injury in TAFRO syndrome was investigated in the present case.