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一名年轻女孩患急性髓系白血病,伴有纵隔粒细胞肉瘤,侵犯心包并导致上腔静脉综合征。

Acute myeloid leukemia in a young girl presenting with mediastinal granulocytic sarcoma invading pericardium and causing superior vena cava syndrome.

作者信息

Tsai Ming-Horng, Yang Chao-Ping, Chung Hung-Tao, Shih Lee-Yung

机构信息

Division of Pediatric Hematology/Oncology, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

出版信息

J Pediatr Hematol Oncol. 2009 Dec;31(12):980-2. doi: 10.1097/MPH.0b013e3181b86ff3.

DOI:10.1097/MPH.0b013e3181b86ff3
PMID:19956024
Abstract

A 1-year-4-month-old girl who presented with pericardial effusion and superior vena cava (SVC) syndrome caused by a mediastinal mass was later proved to be a case of acute myeloid leukemia (AML) with mixed-lineage leukemia-gene translocation. The unusual presentation and the giant blasts with basophilic vacuolated cytoplasm had led to initial misdiagnosis of mediastinal lymphoma. She developed progressive SVC syndrome, unresolved pericardial effusion, and extensive leukemia cutis after initial induction therapy. She died soon after second-course chemotherapy. To our knowledge, this is the first reported case of childhood acute myeloid leukemia presenting with mediastinal granulocytic sarcoma causing pericardium invasion and SVC syndrome.

摘要

一名1岁4个月大的女童因纵隔肿块导致心包积液和上腔静脉综合征,后来被证实是一例伴有混合谱系白血病基因易位的急性髓系白血病(AML)。其不寻常的表现以及具有嗜碱性空泡状细胞质的巨大原始细胞导致最初被误诊为纵隔淋巴瘤。在初始诱导治疗后,她出现了进行性上腔静脉综合征、未消退的心包积液和广泛的白血病性皮肤浸润。在第二疗程化疗后不久她便去世。据我们所知,这是首例报道的儿童急性髓系白血病表现为纵隔粒细胞肉瘤并导致心包侵犯和上腔静脉综合征的病例。

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