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 overactivat...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.展开更多
Rituximab maintenance(RM)therapy following successful induction has recently emerged as a highly effective treatment for follicular lymphoma(FL).Randomized trials analyzing the impact of RM compared to observation alo...Rituximab maintenance(RM)therapy following successful induction has recently emerged as a highly effective treatment for follicular lymphoma(FL).Randomized trials analyzing the impact of RM compared to observation alone have demonstrated a significantly better outcome in terms of progression-free survival(but not overall survival)in patients(pts)who received as first-line treatment single-agent rituximab,standard chemotherapy(CVP)and recently also immunochemotherapy(R-CHOP,R-CVP or R-FND),as shown by preliminary results of the PRIMA trial.Also in the setting of relapsed disease,RM has shown significant benefit either after chemotherapy or immunochemotherapy.RM has been generally well tolerated,and treated pts developed only mild toxicity,mainly a small increased rate of neutropenia,hypogammaglobulinaemia and self-limiting upper-respiratory tract infections.Moreover,no cumulative or unexpected toxicities were observed and quality of life was not affected.These data have established RM therapy as an important part of multi-modal therapeutic strategies in patients affected by FL.展开更多
文摘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.
文摘Rituximab maintenance(RM)therapy following successful induction has recently emerged as a highly effective treatment for follicular lymphoma(FL).Randomized trials analyzing the impact of RM compared to observation alone have demonstrated a significantly better outcome in terms of progression-free survival(but not overall survival)in patients(pts)who received as first-line treatment single-agent rituximab,standard chemotherapy(CVP)and recently also immunochemotherapy(R-CHOP,R-CVP or R-FND),as shown by preliminary results of the PRIMA trial.Also in the setting of relapsed disease,RM has shown significant benefit either after chemotherapy or immunochemotherapy.RM has been generally well tolerated,and treated pts developed only mild toxicity,mainly a small increased rate of neutropenia,hypogammaglobulinaemia and self-limiting upper-respiratory tract infections.Moreover,no cumulative or unexpected toxicities were observed and quality of life was not affected.These data have established RM therapy as an important part of multi-modal therapeutic strategies in patients affected by FL.