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首页> 外文期刊>European Journal of Haematology >Coexistence of sickle cell disease and severe congenital neutropenia: First impressions can be deceiving
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Coexistence of sickle cell disease and severe congenital neutropenia: First impressions can be deceiving

机译:镰状细胞病和严重的先天性中性粒细胞减少症并存:第一印象可能是欺骗

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摘要

We report an Omani family in whom the propositus had a rare coexistence of sickle cell disease and severe congenital neutropenia associated with a mutation in ELANE. In contrast to his siblings with sickle cell disease, the severity of HbSS-associated complications such as painful crises and acute chest syndrome was significantly reduced. His course of the disease had markedly worsened after initiating G-CSF therapy. These clinical observations suggest that neutropenia may ameliorate inflammatory responses and thus display a modulating factor with respect to the clinical course of sickle cell disease.
机译:我们报告了一个阿曼家族,其中的性命中有罕见的镰状细胞疾病和严重的先天性中性粒细胞减少症并存,与ELANE突变相关。与他患有镰状细胞病的兄弟姐妹相反,HbSS相关并发症的严重性(例如痛苦的危机和急性胸腔综合症)显着降低。开始使用G-CSF治疗后,他的病程明显恶化。这些临床观察表明,嗜中性白血球减少症可以改善炎症反应,因此在镰状细胞疾病的临床过程中表现出调节因子。

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