Acute kidney injury due to thin basement membrane disease mimicking Deferasirox nephrotoxicity: a case report

• Published in: BMC nephrology 2018-12, Vol.19 (1), p.363-6, Article 363
• Main Authors: Oda, Keiko, Katayama, Kan, Tanoue, Akiko, Murata, Tomohiro, Hirota, Yumi, Mizoguchi, Shoko, Hirabayashi, Yosuke, Ito, Takayasu, Ishikawa, Eiji, Dohi, Kaoru, Ito, Masaaki
• Format: Article
• Language: English
• Subjects: Acute kidney injury; Acute Kidney Injury - etiology; Basement membranes; Biopsy; Blood; Case Report; Case reports; Creatinine; Deferasirox - adverse effects; Deferasirox - gross hematuria - myelodysplastic syndrome - thin basement membrane disease; Diagnosis, Differential; Erythrocytes; Fanconi syndrome; Fever; Gangrene; Glomerular Basement Membrane - pathology; Hematuria; Hematuria - etiology; Hemodialysis; Hospitalization; Humans; Iron; Iron Chelating Agents - adverse effects; Kidney Tubules - pathology; Kidneys; Laboratories; Male; Middle Aged; Mimicry; Mutation; Myelodysplastic syndrome; Myelodysplastic syndromes; Necrosis - diagnosis; Nephritis; Nephrology; Proteins; Toxicity; Urinalysis
• ISSN: 1471-2369; 1471-2369
• DOI: 10.1186/s12882-018-1180-2

Although the renal toxicity of Deferasirox, an oral iron chelator, has been reported to be mild, there have been reports of acute interstitial nephritis or Fanconi syndrome due to this agent. Thin basement membrane disease (TBMD) is a hereditary disease characterized primarily by hematuria, with gross hematuria also observed in about 7% of cases. We herein report a case of TBMD that presented with acute kidney injury and gross hematuria during treatment with Deferasirox. The patient was a 63-year-old man who had been diagnosed with myelodysplastic syndrome 6 years ago. He had started taking Deferasirox at 125 mg due to post-transfusion iron overload 6 months ago. Deferasirox was then increased to 1000 mg three months ago. When the serum creatinine level increased, Deferasirox was reduced to 500 mg three weeks before hospitalization. Although the serum creatinine level decreased once, he developed a fever and macroscopic hematuria one week before hospitalization. The serum creatinine level increased again, and Deferasirox was stopped four days before hospitalization. He was admitted for the evaluation of acute kidney injury and gross hematuria. Treatment with temporary hemodialysis was required, and a kidney biopsy was performed on the eighth day of admission. Although there was no major abnormality in the glomeruli, the leakage of red blood cells into the Bowman's space was observed. Erythrocyte cast formation was observed in the tubular lumen, which was associated with acute tubular necrosis. The results of an electron microscopic study were compatible with TBMD. Although Deferasirox is known to be nephrotoxic, gross hematuria is relatively rare. When we encounter a case of acute kidney injury with gross hematuria during treatment with Deferasirox, TBMD should be considered as a possible cause of gross hematuria.