Phan Thu Dang Anh, Phung Bao Gia, Duong Tu Thanh, Hoang Vu Anh, Ngo Dat Quoc, Trinh Nguyen Dinh The, Tran Tung Thanh
Department of Pathology, University of Medicine and Pharmacy at Ho Chi Minh City, Ho Chi Minh City, Vietnam.
Department of Pathology, City Children Hospital, Ho Chi Minh City, Vietnam.
J Pathol Transl Med. 2021 Mar;55(2):112-117. doi: 10.4132/jptm.2020.11.30. Epub 2021 Jan 27.
Langerhans cell histiocytosis (LCH) is more common in children than adults and involves many organs. In children, the BRAF V600E mutation is associated with recurrent and high-risk LCH.
We collected paraffin blocks of 94 pediatric LCH patients to detect BRAF V600E mutation by sequencing. The relationship between BRAF V600E status and clinicopathological parameters were also critically analyzed.
BRAF V600E mutation exon 15 was detected in 45 cases (47.9%). Multiple systems LCH showed a significantly higher BRAF V600E mutation rate than a single system (p=.001). No statistical significance was evident for other clinical characteristics such as age, sex, location, risk organs involvement, and CD1a expression.
In Vietnamese LCH children, the proportion of BRAF V600E mutational status was relatively high and related to multiple systems.
朗格汉斯细胞组织细胞增多症（LCH）在儿童中比成人更常见，且累及多个器官。在儿童中，BRAF V600E突变与复发性和高危LCH相关。
我们收集了94例儿童LCH患者的石蜡块，通过测序检测BRAF V600E突变。还对BRAF V600E状态与临床病理参数之间的关系进行了严格分析。
45例（47.9%）检测到BRAF V600E突变外显子15。多系统LCH的BRAF V600E突变率显著高于单系统（p = 0.001）。年龄、性别、部位、累及风险器官和CD1a表达等其他临床特征无明显统计学意义。
在越南LCH儿童中，BRAF V600E突变状态的比例相对较高，且与多系统有关。
