再生障碍性贫血-南非背景

T. Ramsamy, C. Solomon, G. Demetriou
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摘要

背景:南非缺乏记录再生障碍性贫血(AA)的各种原因以及对免疫抑制治疗的反应的数据。目的:了解约翰内斯堡学术医院(CMJAH) AA病例的绝对数量、可能的病因及对免疫抑制治疗的反应。方法:回顾性分析2014年1月至2019年6月在CMJAH确诊的所有AA病例。结果:研究期间共发生35例AA。65%的研究样本没有明确的AA原因,被定义为特发性。12例患者(34%)有明确的AA继发原因;其中包括接触药物和毒素、怀孕、病毒感染和自身免疫疾病。所有患者均接受联合免疫抑制治疗。这些患者都没有接受过干细胞移植。18%的患者对治疗有完全反应,49%有部分反应,29%对治疗难治。4%的患者在治疗后复发。结论:AA是一种罕见的疾病,死亡率高。所有可能的继发原因应通过详细的病史和相关调查来寻找。所有患者必须按照推荐的治疗方案进行治疗,并应停止接触不良药物/毒素。应尽一切努力改善患者随访,以便建立关于死亡率和长期并发症的具体数据。
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Aplastic Anaemia—A South African Context
Background: There is a paucity of data in South Africa documenting the various causes of aplastic anaemia (AA), as well as the response to immunosuppressive therapy. Objective: To determine the absolute number of AA cases, possible causes and response to immunosuppressive therapy at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH). Method: A retrospective cross-sectional analysis was conducted of all confirmed cases of AA at the CMJAH for the period of January 2014 to June 2019. Results: There were 35 cases of AA for the given study period. Sixty-five percent of the study sample had no identifiable cause for AA and were defined as idiopathic. Twelve patients (34%) had identifiable secondary causes for AA; these included exposure to drugs and toxins, pregnancy, viral infection and auto-immune conditions. All patients were treated with combination immunosuppressive therapy. None of the patients had received a stem-cell transplant. Eighteen percent of patients had a complete response to therapy, 49% had a partial response whilst 29% were refractory to therapy. Four percent of patients relapsed after treatment. Conclusion: AA is a rare disease with significant mortality. All possible secondary causes should be sought with a detailed patient history and relevant investigations. All patients must be treated with the recommended treatment protocol and exposure to offending drugs/toxins should be terminated. Every effort to improve patient follow-up should be made so as to establish concrete data on mortality and long-term complications.
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