Kidney Week

Abstract: PUB579

A Case of TAFRO Syndrome Associated with Membrano-Proliferative Glomerulopathy and Rescued from Dialysis Therapy by Use of Tocilizumab

Session Information

• Publication Only

Category: Trainee Case Report

• 1202 Glomerular Diseases: Immunology and Inflammation

Authors

• Sekiguchi, Momoko, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Momoko Sekiguchi,

• Kogure, Yuta, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Yuta Kogure,

• Hatano, Minoru, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Minoru Hatano,

• Iwashita, Takatsugu, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Takatsugu Iwashita,

• Shimozato, Yu, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Yu Shimozato,

• Kurosawa, Akira, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Akira Kurosawa,

• Yamasaki, Maiko, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Maiko Yamasaki,

• Ogawa, Tomonari, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Tomonari Ogawa,

• Kanozawa, Koichi, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Koichi Kanozawa,

• Hasegawa, Hajime, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

Hajime Hasegawa,

Introduction

TAFRO syndrome is a rare disease of unknown etiology characterized by Thrombocytopenia, Anasarca, Fever with systemic inflammation, Reticular fibrosis in bone marrow and Organomegaly or lymph node swelling. TAFRO is occasionally accompanied by renal involvement leading to endstage renal failure, however, renal histopathology is rarely confirmed because of life-threatened rapid progression and thrombocytopenia. Here, we report a case of TAFRO syndrome showing membrano-proliferative glomerulopathy-like glomerular findings.

Case Description

An 80-year-old man was admitted to a local hospital because of high fever, anemia, bleeding tendency and systemic edema from 1 month prior to the admission to our hospital. He received RBC transfusion and diuretics administration, however, he rapidly developed renal failure requiring dialysis therapy on the 7th hospital day. After transferred to our hospital, he received 1 g of prednisolone (PSL) for 3 consecutive days with continuous hemodialysis, and TAFRO syndrome was suspected because of systemic inflammation and sever thrombocytopenia (2x10⁴ per µL). The diagnosis was confirmed by lymph node biopsy showing hyaline-vascular finding similar to Castelman’s disease and remarkable elevation of interleukin-6 and vascular endothelial growth factor. His clinical condition was apparently ameliorated by maintaining administration of PSL 30 mg a day, and his dialysis therapy was discontinued at the 67th hospital day. At that time, renal biopsy revealed membrano-proliferative glomerulopathy with slight tubulo-interstitial damage. Following PSL withdrawal, his renal function and proteinuria was worsened again. By this reason, Tocilizumab 400 mg was administered and repeated every 2 weeks. After start of Tocilizumab, his clinical condition was ameliorated again and stabilized. He was finally discharged on the 152nd hospital day.

Discussion

TAFRO syndrome usually become sever rapidly without renal biopsy, and clinical benefit of Tocilizumab is not generally established. Our case might be a valuable index to consider the mechanism of renal involvement and the clinical management.