骨髓增生异常综合征的髓外造血:系统文献综述

IF 0.9 Q4 HEMATOLOGY Hemato Pub Date : 2022-09-19 DOI:10.3390/hemato3030039
Chen Wang, Yiyun Shi
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引用次数: 0

摘要

髓外造血很少见于骨髓增生异常综合征患者,其临床特征不明确。在这里,我们系统地回顾了已发表的文献,总结了骨髓增生异常综合征患者经活检证实的髓外造血的临床表现、治疗和长期预后。我们纳入了41例患者,环状铁母细胞是最常见的骨髓增生异常亚型(30.6%)。髓外造血因局部受压而出现典型症状,常累及肝脏或脾脏(36.6%)或椎旁区(24.4%)。值得注意的是,环状铁母细胞主要见于非肝脾受累的患者(38.5% vs. 6.7%, p = 0.034)。必要时的干预措施通常包括手术(36.8%)或放疗(13.2%),这导致55.5%的患者症状改善。当前队列的中位总生存期为7个月。目前的研究证实髓外造血作为骨髓增生异常综合征的并发症是罕见的;然而,其对系统性现代治疗的反应结果需要进一步研究。
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Extramedullary Hematopoiesis in Myelodysplastic Syndromes: A Systematic Literature Review
Extramedullary hematopoiesis is rarely seen in patients with myelodysplastic syndromes, and its clinical characterizations are not well-defined. Here, we systematically reviewed the published literature to summarize the clinical manifestations, treatments, and long-term outcomes of biopsy-proven extramedullary hematopoiesis in patients with myelodysplastic syndromes. We included 41 patients, and ring sideroblasts were the most common myelodysplastic subtype (30.6%). Extramedullary hematopoiesis was typically symptomatic on presentation due to local compression, frequently involving the liver or spleen (36.6%), or the paravertebral region (24.4%). Notably, ring sideroblasts were predominantly seen in patients with non-hepatosplenic involvement (38.5 vs. 6.7%, p = 0.034). Interventions, when required, usually included surgery (36.8%) or radiation (13.2%), which led to symptomatic improvement in 55.5% of patients. The median overall survival of the current cohort was 7 months. The current study confirms the rarity of extramedullary hematopoiesis as a complication of myelodysplastic syndromes; however, its outcomes in response to systemic modern therapies require further investigation.
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来源期刊
CiteScore
1.30
自引率
0.00%
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0
审稿时长
11 weeks
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