[1]张 鼎,赵 斌.朗格汉斯组织细胞增生症的诊治进展[J].医学信息,2019,(19):35-38.[doi:10.3969/j.issn.1006-1959.2019.19.012]
 ZHANG Ding,ZHAO Bin.Progress in the Diagnosis and Treatment of Langerhans Cell Histiocytosis[J].Medical Information,2019,(19):35-38.[doi:10.3969/j.issn.1006-1959.2019.19.012]
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朗格汉斯组织细胞增生症的诊治进展()
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医学信息[ISSN:1006-1959/CN:61-1278/R]

卷:
期数:
2019年19期
页码:
35-38
栏目:
综述
出版日期:
2019-10-01

文章信息/Info

Title:
Progress in the Diagnosis and Treatment of Langerhans Cell Histiocytosis
文章编号:
1006-1959(2019)19-0035-04
作者:
张 鼎1赵 斌2
(1.福建中医药大学,福建 福州 350108;2.厦门市中医院,福建 厦门 361009)
Author(s):
ZHANG Ding1ZHAO Bin2
(1.Fujian University of Traditional Chinese Medicine,Fuzhou 350108,Fujian,China;2.Xiamen Hospital of Traditional Chinese Medicine,Xiamen361009,Fujian,China)
关键词:
朗格汉斯组织细胞增生症单核-巨噬细胞异常增生炎性髓样肿瘤
Keywords:
Langerhans cell histiocytosisMononuclear-macrophage dysplasiaInflammatory myeloid tumor
分类号:
R55
DOI:
10.3969/j.issn.1006-1959.2019.19.012
文献标志码:
A
摘要:
朗格汉斯组织细胞增生症(LCH)是一种少见的、病因尚不明确的单核-巨噬细胞异常增生性疾病,其临床表现具有多样性,累及多个系统,且发病机制复杂,目前主要认为是炎性髓样肿瘤,治疗上仍以联合化疗为主,随着靶向药物及骨髓移植应用逐渐增多,其并发症及不良反应需进一步解决。对于单系统受累LCH 患者预后较佳,临床报道治愈病例较多。而多系统受累LCH患者预后不佳,病死率高。本文就近年来有关LCH的发病机制、临床表现、诊断与治疗及预后进展进行综述。
Abstract:
Langerhans cell histiocytosis (LCH) is a rare mononuclear-macrophage dysplasia with unclear etiology. Its clinical manifestations are diverse, involving multiple systems, and the pathogenesis is complex. At present, it is mainly considered to be an inflammatory myeloid tumor. The treatment is still based on combined chemotherapy. With the increasing application of targeted drugs and bone marrow transplantation, its complications and adverse reactions need to be further resolved. The prognosis of LCH patients with single system involvement is better, and more cases are reported in clinical reports. The multi-system involvement of LCH patients has a poor prognosis and a high mortality rate. This article reviews recent advances in the pathogenesis, clinical manifestations, diagnosis and treatment, and prognosis of LCH.

参考文献/References:

[1]Horibe K,Saito AM,Takimoto T,et al.Incidence and survival rates of hematological malignancies in Japanese children and adolescents(2006-2010):based on registry data from the Japanese Society of Pediatric Hematology[J].Int J Hematol,2013,98(1):74-88. [2]Swerdlow SH,Campo E,Pileri SA,et al.WHO classification of tumours of haematopoietic and lymphoid tissues.In:Weiss LM,Jaffe R,Facchetti F,eds.WHO classification of Tumours of Haematopoietic and Lymphoid Tissues[J].Blood,2016,127(20):2375. [3]Badalian-Very G,Vergilio J A,Degar B A,et al.recurrent BRAF mutations in Langerhans cell histiocytosis[J].Blood,2010,116(11):1919-1923. [4]Ozer Erdener,Sevinc Akin,Ince Dilek,et al.BRAF V600E Mutation: A Significant Biomarker for Prediction of Disease Relapse in Pediatric Langerhans Cell Histiocytosis[J].Pediatric and developmental pathology,2019,36(1):69-86. [5]Brown NA,Furtado LV,Betz BL,et al.High prevalence of somatic MAP2K1 mutations in BRAF V600E-negative Langerhans cell histiocytosis[J].Blood,2014,124(10):1655-1658. [6]Emile JF,Abla O,Fraitag S,et al.Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages[J].Blood,2016,127(22):2672-2681. [7]Tazi A,de Margerie C,Naccache JM,et al.The natural history of adult pulmonary Langerhans cell histiocytosis:a prospective multicentre study[J].Orphanet J Rare Dis,2015,10(3):30. [8]李建红,李萍,唐鹏跃,等.儿童朗格汉斯细胞组织细胞增生症20例临床分析[J].中国皮肤性病学杂志,2018,32(6):650-653. [9]赵丽,赵林胜,苏海辉,等.以皮疹为首发症状的儿童朗格汉斯细胞组织细胞增生症19例临床病理分析[J].临床与实验病理学杂志,2018,34(5):550-552. [10]张丽丽,刘玲,武强,等.15例儿童朗格汉斯细胞组织细胞增生症病理诊断及鉴别诊断[J].河北医科大学学报,2018,39(7):817-821. [11]KobayashiM,TojoA.Langerhans cell histiocytosis in adults:Advances in pathophysiology and treatment[J].Cancer Science,2018,109(12):3707-3713. [12]杨芳,王晓莉.儿童朗格汉斯细胞组织细胞增生症3例并文献复习[J].福建医药杂志,2018,40(5):40-44,182. [13]Tazi A,Lorillon G,Haroche J,et al.Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis:a multicenter retrospective study[J].Orphanet J Rare Dis,2017,12(1):95. [14]刘玉洁,刘炜,陶箐,等.30例高危多系统性朗格汉斯细胞组织细胞增生症的临床病理特征并文献复习[J].临床与病理杂志,2018,38(10):2094-2101. [15]Héritier S,Hélias-Rodzewicz Z,Lapillonne H,et al.Circulating cellfree BRAFV600E as a biomarker in children with Langerhans cell histiocytosis[J].Br J Haematol,2017,178(3):457-467. [16]Simko SJ,McClain KL,Allen CE.Up-front therapy for LCH:is it time to test an alternative to vinblastine/prednisone[J].Br J Haematol,2015,169(2):299-301. [17]Craig Erker,Paul Harker-Murray,Julie-An Talano.Usual and unusual manifestations of familial hemophagocytic lymphohistiocytosis and langerhans cell histiocytosis[J].The Pediatric Clinics of North America,2017,64(1):91-109. [18]Berres ML,Lim KP,Peters T,et al.BRAF-V600E expression in precursor versus differentiated dendritic cells defines clinically distinct LCH risk groups[J].J Exp Med,2015,212(2):281. [19]Derenzini E,Stefoni V,Pellegrini C,et al.High efficacy of the MACOP-B regimen in the treatment of adult Langerhans cell histiocytosis,a 20 year experience[J].BMC Cancer,2015,15(1):879. [20]Veys PA,Nanduri V,Baker KS,et al.Haematopoietic stem cell transplantation for refractory langerhans cell histiocytosis:outcome by intensity of conditioning[J].Br J Haemato,2015,169(5):711-718. [21]Gabalec Filip,imkoviMartin,Zavelová Alběta,et al.Treatment of Multifocal Multisystem BRAF Positive Langerhans Cell Histiocytosis with Cladribine,Surgery and Allogenic Stem Cell Transplantation[J].Acta medica,2017,60(4):152-156. [22]闫美玲,张萌,黄琳,等.BRAF V600E抑制剂治疗小儿朗格罕斯组织细胞增生症的临床分析[J].中国新药杂志,2019,28(7):886-890. [23]Diamond EL,Subbiah V,Lockhart AC,et al.Vemurafenib for BRAF V600-Mutant Erdheim-Chester Disease and Langerhans CellHistiocytosis:analysis of data from the histology-independent,phase 2,open-label VEBASKET Study[J].JAMA Oncol,2018(4):384-388. [24]Papapanagiotou M,Griewank KG,Hillen U,et al.Trametinibinduced remission of an MEK1-mutated langerhans cell histiocytosis[J].JCO Precis Oncol,2017(1):1-5. [25]Chow TW,Leung WK,Cheng FWT,et al.Late outcomes in children with Langerhans cell histiocytosis[J].Archives of Disease in Childhood,2017,102(9):830-835.

更新日期/Last Update: 2019-10-01