Dr Martin Klein1,2, Dr Steven Thien Nguyen1,2
1South West Renal Services, Warrnambool Base Hospital, South West Healthcare, Warrnambool, Australia, 2Department of Nephrology, St Vincent's Hospital Melbourne, Fitzroy, Australia
Biography:
Steven is a first year General and Acute Care Medicine Advanced Trainee. He hopes to complete dual training in Nephrology and General Medicine. He has been focusing on developing a diverse range of skills to best serve his patients as he continues to work towards Nephrology training.
Background:
Thrombotic microangiopathy (TMA) describes a group of disorders hallmarked by endothelial injury precipitating platelet activation, micro-thromboses, microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, resulting in end organ damage most commonly affecting the central nervous system and kidneys. TMA includes thrombotic thrombocytopenic purpura (TTP), classic hemolytic-uremic syndrome (HUS) and atypical hemolytic-uremic syndrome (aHUS). Secondary TMA describes disease associated with a heterogenous group of conditions including autoimmune disease, drug-induced, most commonly calcineurin inhibitors and chemotherapy, solid organ and hematopoietic stem cell transplantation and malignancy. Cancer-associated TMA is rare and the current literature reports associations with adenocarcinomas of the lung, breast and prostate as well as a few reports of metastatic melanoma. There is growing evidence for the role of eculizumab in the management of malignancy associated secondary TMA.
Case Presentation:
We report a case of a 69-year-old man presenting with stage 3 acute kidney injury requiring hemodialysis. Laboratory testing revealed schistocytes on blood film and a profound thrombocytopenia, consistent with a MAHA. The patient’s renal biopsy demonstrated evidence of TMA. He was subsequently diagnosed with a metastatic prostate adenocarcinoma. He was treated with androgen deprivation therapy, and eculizumab with rapid hematologic and renal recovery. The patient no longer required dialysis four weeks after his initial dose of eculizumab. There was sustained remission at five months follow up.
Conclusion:
Eculizumab is generally well tolerated and a reasonable therapeutic option in the management of TMA, particularly in patients with severe disease manifestations. Further investigation is required to further delineate where eculizumab fits amongst standard therapy for secondary TMA.