Aplastic Anemia in the Hematology Department of the University Hospital of Brazzaville: Epidemiological, Diagnostic and Therapeutic Aspects  

作　　者：Firmine Olivia Galiba Atipo-Tsiba Rochelle Délice Ngakosso Onanga Clément Pacha Mikia Claude Hermione Ibovi Haddouce Ocini Lydie Ngolet Alexis Elira Dokekias Firmine Olivia Galiba Atipo-Tsiba;Rochelle Délice Ngakosso Onanga;Clément Pacha Mikia;Claude Hermione Ibovi Haddouce;Ocini Lydie Ngolet;Alexis Elira Dokekias(Hematology Department, University Hospital of Brazzaville, Brazzaville, Republic of the Congo;Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Republic of the Congo)

机构地区：[1]Hematology Department, University Hospital of Brazzaville, Brazzaville, Republic of the Congo [2]Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Republic of the Congo

出　　处：《Open Journal of Blood Diseases》2024年第4期109-118,共10页血液病期刊(英文)

摘　　要：Aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and bone marrow failure. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, outcomes have improved considerably, with 5-year survival reported to be 70% - 90%. In Congo, contemporary data on survival are lacking. We performed a retrospective study to describe the epidemiological, diagnostic, and therapeutic characteristics of patients with AA diagnosed in the clinical hematology department of the University Hospital of Brazzaville from 2017 to 2023. Chemically induced aplasia was excluded from the study. The CAMITTA criteria were used to classify the severity of AA. In total, 45 confirmed cases were identified, and 35 files provided sufficient data for the descriptive study. The median age was 26 years (range: 1 - 50). Adults made up 75% of the study population. The sex ratio was 1.05 (0.5 in children and 1.17 in adults). One case of AA was secondary to treatment with imatinib mesylate;the other cases (97.1%) were idiopathic. Pancytopenia was present in all patients. Moderate, severe, and very severe AA represented 11.4%, 74.3%, and 14.3% of cases, respectively. Severe and very severe forms were more frequent in adults (77.4% vs. 22.6%) and in men. Nine patients (26%) received cyclosporine monotherapy. Only one received treatment regularly and obtained the only favorable response. No patient received ATG or eltrombopag. Hemorrhagic syndrome was the most common cause of death (4 out of 6 cases) due to the unavailability of platelet concentrates. Eighteen patients (51.4% of cases) were lost to follow-up. The median follow-up was 28.7 (1 - 96) months. In conclusion, the prognosis of AA remains poor and could be improved with affordable immunosuppressive treatments, availability of platelet concentrates, and implementation of allogeneic bone marrow transplantation.Aplastic anemia (AA) is a rare, life-threatening disease characterized by pancytopenia and bone marrow failure. However, since the introduction of immunosuppressive therapy and allogeneic stem cell transplantation, outcomes have improved considerably, with 5-year survival reported to be 70% - 90%. In Congo, contemporary data on survival are lacking. We performed a retrospective study to describe the epidemiological, diagnostic, and therapeutic characteristics of patients with AA diagnosed in the clinical hematology department of the University Hospital of Brazzaville from 2017 to 2023. Chemically induced aplasia was excluded from the study. The CAMITTA criteria were used to classify the severity of AA. In total, 45 confirmed cases were identified, and 35 files provided sufficient data for the descriptive study. The median age was 26 years (range: 1 - 50). Adults made up 75% of the study population. The sex ratio was 1.05 (0.5 in children and 1.17 in adults). One case of AA was secondary to treatment with imatinib mesylate;the other cases (97.1%) were idiopathic. Pancytopenia was present in all patients. Moderate, severe, and very severe AA represented 11.4%, 74.3%, and 14.3% of cases, respectively. Severe and very severe forms were more frequent in adults (77.4% vs. 22.6%) and in men. Nine patients (26%) received cyclosporine monotherapy. Only one received treatment regularly and obtained the only favorable response. No patient received ATG or eltrombopag. Hemorrhagic syndrome was the most common cause of death (4 out of 6 cases) due to the unavailability of platelet concentrates. Eighteen patients (51.4% of cases) were lost to follow-up. The median follow-up was 28.7 (1 - 96) months. In conclusion, the prognosis of AA remains poor and could be improved with affordable immunosuppressive treatments, availability of platelet concentrates, and implementation of allogeneic bone marrow transplantation.

关 键 词：Aplastic Anemia Blood Transfusion Bone Marrow Transplantation CYCLOSPORIN CORTICOSTEROIDS Immunosuppressive Therapy 

分 类 号：R55[医药卫生—血液循环系统疾病]