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[恶性嗜酸性粒细胞增多综合征。1例罕见病例报告(作者译)]

[Malignant hypereosinophilic syndrome. Report of an exceptional case (author's transl)].

作者信息

Pardo-Peret P, Sans-Sabrafen J, Buxó Costa J, Rosell Costa R, Woessner S

出版信息

Med Clin (Barc). 1979 Mar 10;72(5):189-94.

PMID:431184
Abstract

The case of a 43-year-old man with a highly malignant hypereosinophilic syndrome is reported. The condition is classified as such according to Hardy and Anderson's criteria, accepted by many other authors. Other diseases of known etiology which may present high levels of eosinophils in the peripheral blood, such as parasitosis, allergies, neoplasias, collagenosis, etc., were discounted beforehand. The difficulties in distinguishing between these diseases are discussed; they are often accompanied by clinical manifestations which also arise in very different conditions including eosinophilic leukemia, Engfeldt and Zetterström's eosinophilic collagenosis, Löffler's fibroplastic endocarditis, etc. A particularly striking feature of this condition is the formation of large tumor masses of mature eosinophils. They begin in various bones, which they destroy almost completely, and invade the surrounding tissues, destroying them as well. These tumors act similarly to malignant eosinophilic myelocytomas, a fact which has not been reported previously in the literature as far as we know. Although the eosinophils act as though they were neoplastic, they maintain the characteristics of mature cells, both cytomorphologically and ultrastructurally as well as cytochemically (consistently chloroacetate esterase negative). The tendency to diagnose eosinophilic leukemia solely on the basis of the malignancy of the condition and a tissue infiltration of eosinophils without determining the existence of cytologic and/or cytochemical anomalies of the cells showing them to be leukemic is discussed. The authors were unable to find any reports in the literature in which the eosinophils were presented with unmistakeably blastic cellular characteristics. Various nosologic considerations are offered.

摘要

报告了一例43岁男性的高度恶性高嗜酸性粒细胞综合征病例。根据哈迪(Hardy)和安德森(Anderson)的标准,该病症被归为此类,许多其他作者也认可这一标准。事先排除了其他已知病因的疾病,这些疾病在外周血中可能出现高水平嗜酸性粒细胞,如寄生虫病、过敏、肿瘤、胶原病等。讨论了区分这些疾病的困难;它们通常伴有在包括嗜酸性粒细胞白血病、恩费尔特(Engfeldt)和泽特斯特伦(Zetterström)嗜酸性胶原病、吕弗勒(Löffler)纤维增生性心内膜炎等非常不同的病症中也会出现的临床表现。这种病症的一个特别显著特征是形成大量成熟嗜酸性粒细胞的肿瘤块。它们始于各种骨骼,几乎将其完全破坏,并侵入周围组织,也将其破坏。这些肿瘤的行为类似于恶性嗜酸性粒细胞髓细胞瘤,据我们所知,这一事实此前在文献中尚未有报道。尽管嗜酸性粒细胞表现得好像是肿瘤性的,但它们在细胞形态学、超微结构以及细胞化学方面(始终氯乙酸酯酶阴性)都保持成熟细胞的特征。讨论了仅根据病症的恶性程度和嗜酸性粒细胞的组织浸润来诊断嗜酸性粒细胞白血病,而不确定显示它们为白血病的细胞是否存在细胞学和/或细胞化学异常的倾向。作者在文献中未能找到任何将嗜酸性粒细胞呈现出明确的原始细胞特征的报道。文中提出了各种疾病分类学上的考量。

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