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Clonal Hypereosinophilic Syndrome: Two Cases Report in Black Men from Sub-Saharan Africa and Literature Reviews

机译:克隆性嗜酸性粒细胞增多综合征:撒哈拉以南非洲黑人两例报道及文献综述

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The first case is about a man of 60 years old suffering of hypereosinophilic syndrome (HES) developed since 1998. He presented chronic cough, insomnia, and negative parasitical test. We observed hypereosinophilia and fibroblastic hyperplasia at the bone marrow biopsy. Initially, hydroxyurea andα-interferon treatment failed. We proposed to him imatinib mesylate in May 2003. The FIP1L1-PDGFRA gene was detected. The second case is about a man of 34 years old seen in March 2002. First investigation concluded to CML. Progressively, eosinophil cells increased, and complications occurred as oedema syndrome, dyspnoea, and parietal chronic endocarditic fibrosis associated with pericarditis. In addition, a bowel obstruction happened and was cured by surgery. Bcr-abl fusion was negative, and FIP1L1-PDGFRA gene was detected after and imatinib mesylate was given. Actually, endocarditic fibrosis decreased. The two patients are in haematological and cytogenetic remission. We concluded that clonal HES is present in Africa, and imatinib mesylate is effective.
机译:第一例是自1998年以来,一名60岁的患有高嗜酸性粒细胞综合症(HES)的男子。他表现出慢性咳嗽,失眠和寄生虫试验阴性。我们在骨髓活检中观察到嗜酸性粒细胞增多和成纤维细胞增生。最初,羟基脲和α-干扰素治疗失败。我们于2003年5月向他提出了甲磺酸伊马替尼的要求。检测到FIP1L1-PDGFRA基因。第二起案件是关于2002年3月发现的一名34岁男子的案件。渐进地,嗜酸性粒细胞增加,并发生并发症,如水肿综合征,呼吸困难和与心包炎有关的顶叶慢性心内膜纤维化。此外,肠梗阻发生并通过手术治愈。给予甲磺酸伊马替尼后,Bcr-abl融合阴性,检测到FIP1L1-PDGFRA基因。实际上,心内膜纤维化减少了。两名患者的血液学和细胞遗传学缓解。我们得出的结论是,非洲存在HES克隆,而甲磺酸伊马替尼有效。

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