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atypical hemolytic uremic syndrome in a Patient with Acute Promyelocytic Leukemia: A Case Report

Case Reports in Clinical Medicine 2023年第2期12卷 37-43页

作者： Pilar Dutari Tantrantan Leonce Adjoumani Marilyne Grinand Olivier Lavelle Moglie Le Quintrec Safia Chebrek Department of Oncohematology Henri Duffaut Hospital Center Avignon France Department of Nephrology Henri Duffaut Hospital Center Avignon France Department of Nephrology Dialysis and Renal Transplantation University Hospital of Lapeyronie Montpellier France

Introduction: Acute Promyelocytic Leukemia (APL) is highly associated with hemostasis alterations. The atypical hemolytic uremic syndrome (aHUS) is a rare type of Thrombotic Microangiopathy (TMA) due to an overactivation of the alternative complement pathway. Case Presentation: A 48-years-old woman was diagnosed with APL and achieved molecular remission after induction therapy. During the second consolidation cycle she presented with TMA. She began treatment with plasma exchange plus corticotherapy but due to aggravation of symptoms Eculizumab was initiated. Thrombotic thrombocytopenic purpura, infections and drug toxicity causes were ruled out. There was no evidence of relapse of the APL. Genetic studies of the hereditary anomalies of the alternative complement pathway were negative and the decision of stopping Eculizumab was made. During maintenance therapy for the APL she presented a severe relapse of the aHUS, requiring dialysis. She re-started treatment with Eculizumab with a progressive hematologic recovery and improvement of renal function. She completed APL treatment without relapse of the leukemia for the moment and continues to be treated with Eculizumab. Conclusion: This is the first published case of coexisting aHUS and APL successfully treated with Eculizumab.

关键词： atypical hemolytic uremic syndrome Acute Promyelocytic Leukemia

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Successful Renal Transplantation in a Patient with atypical hemolytic uremic syndrome Treated with Eculizumab in China

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Chinese Medical Journal 2016年第11期129卷 1379-1381页

作者： Ze-Jia Sun Xin Du Lu-Lu Su Xiao-Dong Zhang Yong Wang Liang Ren Wei Wang Department of Urology Beijing Chaoyang Hospital Capital Medical University Beijing 100020 China

hemolytic uremic syndrome （HUS） is a rare disease characterized by thrombocytopenia and acute renal failure. atypical HUS （aHUS） accounts for approximately 10% of all HUS cases. The pathogenesis of aHUS is mainly associated with gene mutations in complement factor H, complement factor I, and membrane cofactor protein （MCP）. The prognosis of aHUS is generally poor.

关键词： atypical hemolytic uremic syndrome Complement Eculizumab Kidney Transplantation

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Combined liver-kidney transplantation for rare diseases

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World Journal of Hepatology 2020年第10期12卷 722-737页

作者： Mladen Knotek Rafaela Novak Alemka Jaklin-Kekez Anna Mrzljak Department of Medicine Tree Top HospitalHulhumale 23000Maldives Department of Medicine Merkur University HospitalZagreb 10000Croatia School of Medicine University of ZagrebZagreb 10000Croatia Clinic for Pediatric Diseases Helena Zagreb 10000Croatia

Combined liver and kidney transplantation(CLKT)is indicated in patients with failure of both organs,or for the treatment of end-stage chronic kidney disease(ESKD)caused by a genetic defect in the *** aim of the present review is to provide the most up-to-date overview of the rare conditions as indications for *** are major indications for CLKT in ***,in some of them(e.g.,atypical hemolytic uremic syndrome or primary hyperoxaluria),CLKT may be required in adults as *** hyperoxaluria is divided into three types,of which type 1 and 2 lead to *** has been proven effective in renal function replacement,at the same time preventing recurrence of the *** is associated with liver fibrosis in 5%of cases and these patients are candidates for *** alpha 1-antitrypsin deficiency,hereditary C3 deficiency,lecithin cholesterol acyltransferase deficiency and glycogen storage diseases,glomerular or tubulointerstitial disease can lead to chronic kidney *** transplantation as a part of CLKT corrects underlying genetic and consequent metabolic *** atypical hemolytic uremic syndrome caused by mutations in the genes for factor H,successful CLKT has been reported in a small number of ***,for this indication,CLKT has been largely replaced by eculizumab,an anti-C5 *** has been well established to provide immune protection of the transplanted kidney against donor-specific antibodies against class I HLA,facilitating transplantation in a highly sensitized recipient.

关键词： Combined liver-kidney transplantation Methylmalonic aciduria Hereditary complement C3 deficiency Glycogen storage diseases Homozygous protein C deficiency Primary hyperoxaluria atypical hemolytic uremic syndrome Sensitization Donorspecific antibodies

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