伊拉克巴格达骨髓增生异常综合征的概况-临床情况和结果

Waseem F Al-Tameemi, Sabrin Qasim Ibrahim
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摘要

背景:骨髓增生异常综合征(MDS)在诊断和治疗方面是一个挑战,因为它具有异质的表现,对具有白血病转化倾向的各种措施的不可预测的反应。研究目的:回顾伊拉克MDS患者的表现、风险分层和治疗方案,并从反应标准、生活质量和进展方面评估不同治疗方案的结果。患者和方法:从2018年1月到2019年10月,在巴格达不同的血液学中心进行了为期21个月的医院研究。所有被诊断为原发性或治疗相关MDS的患者均被纳入本研究。当应用细胞遗传学和非细胞遗传学评分系统时,每个患者被分为不同的风险组,每个患者的治疗选择。他们观察了他们对不同治疗方式的反应和任何进展的证据。结果:MDS的平均诊断年龄为58.7±贫血是最常见的症状,占患者的68.4%。含有过量blast - ii的MDS占28.8%。多数患者为中危组。50%的MDS患者使用了低甲基化药物,而32.1%的患者接受了最佳治疗。接受HMA治疗的患者达到部分缓解的占7.1%,达到疾病稳定的占25.2%,血液系统改善的占25.2%。21.6例患者死亡,平均生存期为7.7个月。疾病进展为10.8%,白血病转化为7.2%。结论:伊拉克MDS患者年龄较轻,表现较晚,需要标准的危险分层。即使在资源匮乏的情况下,非细胞遗传学评分系统也不能具有同等的应用价值。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Overview of myelodysplastic syndrome in Baghdad, Iraq- clinical picture and outcome
Background: Myelodysplastic syndromes (MDS) can represent a challenge in diagnosis and treatment because of heterogeneous presentation, unpredictable response to variable measures with propensity for leukemic transformation. Aim of study: To review MDS Iraqi patients in term of presentation, risk stratification and treatment options and to assess outcome of different treatment options in term of response criteria, quality of life and progression. Patients and method: a hospital based study conducted over a period of 21 months from January 2018 till October 2019 in different hematology centers in Baghdad. Any patient with primary or therapy related MDS diagnosed were enrolled in this study. Each patient stratified into different risk groups when applying cytogenetic versus non cytogenetic scoring systems, the therapeutic options for every patient. They observed for their response to different therapeutic modalities and any evidence of progression. Results: Mean age at diagnosis for MDS was 58.7± Anemia was the most common presenting symptom representing 68.4% of patients. MDS with excess blasts-II is forming 28.8%. Majority of patients categorized as intermediate risk group. Hypomethylating agents used for 50% of MDS patients while 32.1% received best available therapy. Those on HMA achieve partial remission in 7.1%, 25.2% reached to stable disease, hematological improvement in 25.2%. Death outcome in 21.6 with a mean survival is 7.7 months. Disease progression in 10.8%, 7.2% showed leukemic transformation. Conclusion: Iraqi MDS patients present at younger age with advanced manifestation that needs standard risk stratification. The non-cytogenetic score systems can't be of equivalent application even low resources circumstances.
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