Acute kidney injury induced by thrombotic microangiopathy in a patient with hemophagocytic lymphohistiocytosis

BMC Nephrology

Case Report

Yeong Jin Choi¹  Jong Wook Lee²  Myoung Nam Bae³  Yong-Soo Kim³  Cheol Whee Park³  Chul Woo Yang³  Bum Soon Choi³  Se Jun Park³  Dae Hun Kwak³  Byung Ha Chung⁴ 

[1] Department of Hospital Pathology, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea, 222 Banpodae-ro, 137-040, Seocho-gu, Seoul, Korea;Division of Hematology, Department of Internal Medicine, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea, 222 Banpodae-ro, 137-040, Seocho-gu, Seoul, Korea;Division of Nephrology, Department of Internal Medicine, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea, 222 Banpodae-ro, 137-040, Seocho-gu, Seoul, Korea;Division of Nephrology, Department of Internal Medicine, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea, 222 Banpodae-ro, 137-040, Seocho-gu, Seoul, Korea;Department of Internal Medicine, College of Medicine, Seoul St. Mary’s Hospital, The Catholic University of Korea, 222 Banpodae-ro, 137-040, Seocho-gu, Seoul, Korea;

关键词: Lymphohistiocytosis;    Hemophagocytic;    Thrombotic microangiopathy;    Acute kidney injury;   

DOI  :  10.1186/s12882-015-0217-z

 received in 2015-01-08, accepted in 2015-12-23,  发布年份 2016

来源: Springer

【 摘 要 】

BackgroundHemophagocytic lymphohistiocytosis (HLH) is a fatal clinical syndrome characterized by excessive immune activation and inflammation. It is frequently complicated by acute kidney injury (AKI) that often develops as acute tubular necrosis (ATN). Meanwhile, renal thrombotic microangiopathy (TMA) is a rare pathologic finding that mostly occurs in hemolytic uremic syndrome or thrombotic thrombocytopenic purpura. There are only few reports on TMA developing in patients with HLH. We present here a rare case of TMA associated HLH.Case presentationA 60-year-old woman was admitted for a fever of unknown origin that had persisted for several weeks. She presented with AKI and pancytopenia. Clinical, laboratory and bone marrow biopsy findings met the criteria of HLH. Kidney biopsy showed TMA and minimal ATN, which suggested that the primary cause of AKI was TMA in this case. Because of sustained oliguria, we initiated hemodialysis (HD) and also decided to use chemotherapy composed of dexamethasone, etoposide and cyclosporine for treatment of HLH. Six months after the initiation of chemotherapy, pancytopenia was completely resolved, indicating the resolution of HLH. At the same time, serum creatinine decreased to a normal range without the need for HD, suggesting the resolution of TMA.ConclusionWe report a case of renal TMA associated HLH. This case suggests that renal TMA should be considered as a primary cause of AKI in patients with underlying HLH.

【 授权许可】

CC BY   
© Bae et al. 2016