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伴有ETV6重排的克隆性嗜酸性粒细胞增多症演变为T细胞淋巴母细胞淋巴瘤:一例报告并文献复习

Clonal Hypereosinophilia with ETV6 Rearrangement Evolving to T-Cell Lymphoblastic Lymphoma: A Case Report and Review of the Literature.

作者信息

Moita Filipa, Bogalho Isabel, Alaiz Helena, Parreira Joana, Frade Maria Jesus, Nunes Albertina, Gomes da Silva Maria

机构信息

Hematology Department, Instituto Português de Oncologia de Lisboa, Francisco Gentil, Rua Professor Lima Basto, 1099-213 Lisbon, Portugal.

出版信息

Case Rep Hematol. 2013;2013:652745. doi: 10.1155/2013/652745. Epub 2013 Apr 21.

Abstract

Hypereosinophilia, either clonal or reactive, has been described in association with multiple hematological malignancies. We describe a case of a patient presenting with hypereosinophilia that evolved into T-cell lymphoblastic lymphoma. Complete remission was achieved with chemotherapy; however, hypereosinophilia recurred 5 months later in association with myeloblastic bone marrow infiltration and without evidence of lymphoblastic lymphoma relapse. Cytogenetic analysis of the bone marrow showed a complex translocation involving chromosomes 7, 12, and 16. A rearrangement of ETV6 gene (12p13) was demonstrated by FISH studies, thus confirming the clonality of this population. The association of lymphoblastic lymphoma, eosinophilia, and myeloid hyperplasia has been described in disorders with FGFR1 rearrangements. We hypothesize that other clonal eosinophilic disorders lacking this rearrangement could behave in a similar fashion through different pathogenic mechanisms.

摘要

无论是克隆性还是反应性的嗜酸性粒细胞增多症,均已被描述与多种血液系统恶性肿瘤相关。我们报告一例表现为嗜酸性粒细胞增多症的患者,该病症后来发展为T细胞淋巴母细胞淋巴瘤。化疗实现了完全缓解;然而,5个月后嗜酸性粒细胞增多症复发,伴有髓母细胞骨髓浸润,且无淋巴母细胞淋巴瘤复发的证据。骨髓的细胞遗传学分析显示涉及7号、12号和16号染色体的复杂易位。荧光原位杂交(FISH)研究证实了ETV6基因(12p13)的重排,从而证实了该细胞群的克隆性。在伴有FGFR1重排的疾病中,已描述了淋巴母细胞淋巴瘤、嗜酸性粒细胞增多症和髓样增生之间的关联。我们推测,其他缺乏这种重排的克隆性嗜酸性粒细胞疾病可能通过不同的致病机制表现出类似的行为。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/361e/3654631/21cadc236bed/CRIM.HEMATOLOGY2013-652745.001.jpg

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