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[高嗜酸性粒细胞综合征是一种潜在的恶性疾病吗?]

[Is hypereosinophilic syndrome a potentially malignant condition?].

作者信息

Bellusci M, Nalli G, Mengoni A, Carnevale-Maffè G, Sessa F, Ascari E

机构信息

U.O. II Divisione di Medicina generale, Ospedale S. Chiara, Trento.

出版信息

Recenti Prog Med. 1991 Jan;82(1):19-23.

PMID:2028071
Abstract

The existence of eosinophilic leukemia (EL) has been controversial since it was first described. Recently, some authors have suggested that EL is part of a spectrum of eosinophilic diseases termed hypereosinophilic syndrome (HS). EL diagnosis is very difficult, especially if abnormal chromosome are not present, because HS comprises multiple disease entities of unclear etiology with the common features of prolonged eosinophilia of undetectable cause and organ system dysfunction. We present a case of HS whose findings are consistent with a leukemic process. For two years the patient showed only sharp hypereosinophilia; his clinical course was then marked by signs and symptoms of granulocytic sarcoma (GS), an extramedullary tumor composed of granulocytic precursor cells. GS as a complication of EL was described and in some cases the diagnosis of leukemia was made only on the basis of the GS complication. The formation of eosinophilic GS also suggests a diagnosis of LE in our case of HS. Finally, we always consider HS as a potential malignant disease.

摘要

自首次被描述以来,嗜酸性粒细胞白血病(EL)的存在一直存在争议。最近,一些作者认为EL是被称为嗜酸性粒细胞增多综合征(HS)的一系列嗜酸性粒细胞疾病的一部分。EL的诊断非常困难,尤其是在不存在异常染色体的情况下,因为HS包括多种病因不明的疾病实体,其共同特征是原因不明的长期嗜酸性粒细胞增多和器官系统功能障碍。我们报告一例HS病例,其表现与白血病过程一致。两年来,该患者仅表现为明显的嗜酸性粒细胞增多;随后其临床病程以粒细胞肉瘤(GS)的体征和症状为特征,GS是一种由粒细胞前体细胞组成的髓外肿瘤。GS作为EL的一种并发症已有描述,在某些情况下,仅根据GS并发症做出白血病诊断。嗜酸性粒细胞性GS的形成也提示我们的HS病例应诊断为LE。最后,我们始终将HS视为一种潜在的恶性疾病。

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