An autopsy case of TAFRO syndrome with membranoproliferative glomerulonephritis-like lesions

• Published in: CEN case reports Vol. 8; no. 1; pp. 48 - 54
• Main Authors: Hashimoto, Keiko, Sano, Takashi, Honma, Yukari, Ida, Maoko, Tominaga, Hiroshi, Sawada, Aya, Abe, Tetsuya, Takahashi, Haruka, Shimada, Yoshitaka, Masaki, Takanori, Kamata, Mariko, Naito, Shokichi, Aoyama, Togo, Takeuchi, Yasuo, Akiya, Masashi, Inukai, Madoka, Nakata, Norihiro
• Format: Journal Article
• Language: English
• Published: Singapore Springer Singapore 01.02.2019
• Subjects: Case Report; Medicine; Medicine & Public Health; Nephrology; Urology; TAFRO syndrome; Hypercytokinemia; Membranoproliferative glomerulonephritis-like lesions; Renal dysfunction
• ISSN: 2192-4449; 2192-4449
• DOI: 10.1007/s13730-018-0363-9

TAFRO syndrome (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) is an atypical manifestation of multicentric Castleman’s disease. Although overproduction of interleukin-6, vascular endothelial growth factor, and other cytokines may partially explain the pathophysiology of this rare syndrome, the precise mechanisms underlying the renal dysfunction associated with the condition remain unclear. Here, we describe a case of a 69-year-old male with TAFRO syndrome. He was treated with immunosuppressive agents and his renal function improved. Tapering of immunosuppressive agents resulted in a deterioration of renal function and an elevation of C-reactive protein. After 20 months of treatment, the patient died from tuberculous peritonitis and gastrointestinal bleeding. An autopsy revealed miliary tuberculosis, mediastinal lymphadenopathy, and gastric ulcers. Renal histopathology showed a membranoproliferative glomerulonephritis-like appearance. Almost all glomeruli showed lobular formations with mesangial proliferation and duplication of glomerular capillary walls on light microscopy. Immunofluorescence showed deposition of C1q and IgM along the glomerular capillary walls. Electron microscopy showed mesangial expansion and widening of the subendothelial space with a large number of electron-dense deposits. The glomerular lesions might be characteristic of TAFRO syndrome, and were regarded as the main cause of the patient’s renal dysfunction.