Late renal recovery after treatment over 1 year post-onset in an atypical hemolytic uremic syndrome: A case report

Yusuke Kuroki, Koji Mitsuiki, Kaneyasu Nakagawa, Kazuhiko Tsuruya, Ritsuko Katafuchi, Hideki Hirakata, Toshiaki Nakano

Research output: Contribution to journalArticle

Abstract

Background: Atypical hemolytic uremic syndrome (aHUS) is a life-threatening disease that leads to end-stage kidney disease if only a poor response to plasma exchanges (PEs) or eculizumab therapy is achieved. Case presentation: A 58-year-old Japanese man presented with thrombocytopenia, anemia, and kidney failure requiring dialysis without any underlying disease. A kidney biopsy revealed marked mesangiolysis in all glomeruli, compatible with thrombotic microangiopathy (TMA). Based on the positive anti- factor H antibody and negative result for secondary TMA, we diagnosed him as aHUS. Despite eculizumab administration after eight sessions of PE, neither platelet normalization nor kidney recovery was achieved. Eight months later, we discontinued eculizumab therapy due to anaphylactic reaction. At 15 months after the onset of TMA, his platelet count increased gradually from 40 to 150 × 103/μL with a decreased serum creatinine level and increased urine output, eventually allowing the withdrawal of dialysis therapy. A second kidney biopsy showed mesangial widening compatible with the healing of TMA. Conclusions: This case indicates that aHUS with PEs and eculizumab therapy has the potential for renal recovery even if over 1 year has passed.

Original languageEnglish
Article number236
JournalBMC Nephrology
Volume21
Issue number1
DOIs
Publication statusPublished - Jun 22 2020

All Science Journal Classification (ASJC) codes

  • Nephrology

Fingerprint Dive into the research topics of 'Late renal recovery after treatment over 1 year post-onset in an atypical hemolytic uremic syndrome: A case report'. Together they form a unique fingerprint.

Cite this