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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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