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下丘脑肥胖综合征:CNS+B 细胞淋巴母细胞淋巴瘤的罕见表现。

Hypothalamic obesity syndrome: rare presentation of CNS+ B-cell lymphoblastic lymphoma.

机构信息

Section of Pediatric Hematology/Oncology, Riley Hospital for Children, Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN 46202, USA.

出版信息

Pediatr Blood Cancer. 2012 Nov;59(5):930-3. doi: 10.1002/pbc.24058. Epub 2011 Dec 27.

Abstract

Hypothalamic obesity syndrome can affect brain tumor patients following surgical intervention and irradiation. This syndrome is rare at diagnosis in childhood cancer, but has been reported with relapse of acute lymphoblastic leukemia. Here we present a case of hypothalamic obesity syndrome as the primary presentation of a toddler found to have CNS+ B-cell lymphoblastic lymphoma. Cytogenetic studies on diagnostic cerebrospinal fluid revealed MLL gene rearrangement (11q23). Hyperphagia and obesity dramatically improved following induction and consolidation chemotherapy. We describe a novel presentation of hypothalamic obesity syndrome in CNS B-cell lymphoblastic lymphoma, responsive to chemotherapy.

摘要

下丘脑肥胖综合征可影响接受手术干预和放疗的脑肿瘤患者。这种综合征在儿童癌症的诊断中很少见,但在急性淋巴细胞白血病复发时已有报道。在这里,我们报告了一例以下丘脑肥胖综合征为主要表现的幼儿病例,该幼儿被诊断为中枢神经系统+B 细胞淋巴母细胞淋巴瘤。对诊断性脑脊液的细胞遗传学研究显示 MLL 基因重排(11q23)。诱导和巩固化疗后,贪食和肥胖显著改善。我们描述了中枢神经系统 B 细胞淋巴母细胞瘤中一种新的下丘脑肥胖综合征表现,对化疗有反应。

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11q23/MLL rearrangements in adult acute leukemia.成人急性白血病中 11q23/MLL 重排。
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