Published online Nov 6, 2017. doi: 10.5527/wjn.v6.i6.243
Peer-review started: June 28, 2017
First decision: September 4, 2017
Revised: September 12, 2017
Accepted: November 1, 2017
Article in press: November 1, 2017
Published online: November 6, 2017
A middle age woman who presented with acute kidney injury (AKI) and features of thrombotic microangiopathy (TMA).
TMA, most likely atypical hemolytic uremic syndrome.
Causes of TMA with AKI: hemolytic uremic syndrome, thrombotic thrombocytopenic purpura (primary and secondary causes).
Thrombocytopenia, elevated lactate dehydrogenase, schistocytes on peripheral blood film, acute kidney injury with normal coagulation profile and complement factor I mutation on genetic testing.
Computer tomography to exclude underlying malignancy as secondary causes.
Renal biopsy showing features of TMA with renal cortical necrosis, and acute tubular necrosis.
Plasma exchange, dialysis and eculizumab.
Previous cases of aHUS showing remission with initiation of eculizumab and maintenance of remission despite its discontinuation.
AKI: Acute kidney injury; aHUS: Atypical hemolytic uremic syndrome; CFH: Complement factor H; CFI: Complement factor I; MCP: Membrane co-factor protein; PLEX: Plasma exchange; TMA: Thrombotic microangiopathy; TTP: Thrombotic thrombocytopenic purpura.
aHUS is a serious diagnosis that requires a high index of suspicion in cases presenting with unexplained AKI associated with microangiopathy. Renal benefit of eculizumab may be seen even with late initiation of the drug.