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[血液系统疾病与嗜酸性粒细胞增多症]

[Hematological disorders and hypereosinophilias].

作者信息

Malfuson J-V, Fagot T, Konopacki J, Mangouka L, Souleau B, de Revel T

机构信息

Service d'hématologie clinique, hôpital d'instruction des Armées Percy, 101, avenue Henri-Barbusse, BP406, 92141 Clamart, France.

出版信息

Rev Med Interne. 2009 Apr;30(4):322-30. doi: 10.1016/j.revmed.2008.10.019. Epub 2009 Feb 7.

Abstract

Hematological disorders are the third cause of hypereosinophilia, after allergic and parasitic diseases. Hematological disorders associated with hypereosinophilias can be classified as clonal, reactive or idiopathic, and recently the improvements of cytogenetic, molecular biology and immunology have allowed to revisit numerous cases previously diagnosed as idiopathic hypereosinophilic syndrome. Reactive eosinophilias are mainly associated with lymphoma or abnormal, often clonal T lymphoid population. Clonal eosinophilia is related either to various myeloid malignancies or to a genuine myeloproliferative disorder from the eosinophile lineage, the so-called chronic eosinophilic leukaemia. Chronic eosinophilic leukaemia can be associated with recurrent genes rearrangements involving PDGFRA, PDGFRB and FGFR1 or with clonal abnormalities not yet categorized. Idiopathic hypereosinophilic syndrome remains an exclusive diagnosis in presence of moderate or severe unexplained eosinophilia with target organ damage. The purpose of the diagnostic work-up of hypereosinophilic syndrome is to evidence either an abnormal T cell population or a clonal haematopoiesis. Imatinib mesylate dramatically improves chronic eosinophilic leukaemias associated with PDGFR abnormalities, while corticosteroids are still the main treatment for the other patients. In a near future, advances could arise from identification of new genes involved in clonal eosinophilia or in alternative therapy such as the anti-IL-5 antibodies.

摘要

血液系统疾病是嗜酸性粒细胞增多症的第三大病因,仅次于过敏性疾病和寄生虫病。与嗜酸性粒细胞增多相关的血液系统疾病可分为克隆性、反应性或特发性,最近细胞遗传学、分子生物学和免疫学的进展使得重新审视许多先前被诊断为特发性嗜酸性粒细胞增多综合征的病例成为可能。反应性嗜酸性粒细胞增多主要与淋巴瘤或异常的、通常为克隆性的T淋巴细胞群有关。克隆性嗜酸性粒细胞增多与各种髓系恶性肿瘤或真正的嗜酸性粒细胞系骨髓增殖性疾病(即所谓的慢性嗜酸性粒细胞白血病)有关。慢性嗜酸性粒细胞白血病可与涉及PDGFRA、PDGFRB和FGFR1的复发性基因重排或尚未分类的克隆性异常相关。特发性嗜酸性粒细胞增多综合征在存在中度或重度不明原因的嗜酸性粒细胞增多且伴有靶器官损害时仍是唯一的诊断。嗜酸性粒细胞增多综合征诊断检查的目的是证明存在异常的T细胞群或克隆性造血。甲磺酸伊马替尼可显著改善与PDGFR异常相关的慢性嗜酸性粒细胞白血病,而皮质类固醇仍是其他患者的主要治疗方法。在不久的将来,对参与克隆性嗜酸性粒细胞增多的新基因的鉴定或抗IL-5抗体等替代疗法可能会取得进展。

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