克隆性高嗜酸性粒细胞综合征:撒哈拉以南非洲黑人2例报告及文献综述。

ISRN Hematology Pub Date : 2011-01-01 Epub Date: 2011-03-22 DOI:10.5402/2011/974609
Kodjovi Messie, Ahoefa Vovor, Irenee Messanh Kueviakoe, Levi Kankoe Sallah, Kossi Agbetiafa, Akuete Yvon Segbena
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引用次数: 5

摘要

第一个病例是一名60岁的男性,自1998年以来患有嗜酸性粒细胞增多综合征(HES)。慢性咳嗽,失眠,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纤维母细胞增生。最初,羟基脲和α-干扰素治疗失败。2003年5月,我们向他推荐甲磺酸伊马替尼。检测FIP1L1-PDGFRA基因。第二宗个案涉及一名34岁男子,于2002年3月被发现。首次调查结论为CML。逐渐地,嗜酸性细胞增多,并发水肿综合征、呼吸困难和心包炎相关的壁层慢性心内膜纤维化。此外,发生了肠梗阻,并通过手术治愈。Bcr-abl融合阴性,术后检测FIP1L1-PDGFRA基因,给予甲磺酸伊马替尼。实际上,心内膜纤维化减少了。两例患者血液学和细胞遗传学均有缓解。我们得出结论,克隆HES存在于非洲,甲磺酸伊马替尼是有效的。
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Clonal hypereosinophilic syndrome: two cases report in black men from sub-saharan Africa and literature reviews.

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 and α-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.

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