Langerhans cell histiocytosis in adults: a retrospective, single-center case series
Langerhans cell histiocytosis in adults: a retrospective, single-center case series
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成人朗格汉斯细胞组织细胞增多症：回顾性、单中心病例系列
作者：
S. Chiaravalli;A. Ferrari;L. Bergamaschi;N. Puma;G. Gattuso;G. Sironi;O. Nigro;Virginia Livellara;E. Schiavello;V. Biassoni;M. Podda;C. Meazza;F. Spreafico;M. Casanova;M. Terenziani;R. Luksch;M. Massimino
朗格汉斯细胞组织细胞增生症在成人中很少见，我们对它的诊断和治疗的了解大多来自儿科研究。我们报告了63例连续成人患者（18-76岁）的临床发现和治疗结果，这些患者从1990年到2020年在我们的儿科病房使用与儿童相同的方法进行治疗。41例患者被归类为单系统疾病（SS-LCH），其中34例为单灶性，7例为多灶性，或17例为多系统疾病（MS-LCH），5例为原发性肺（pLCH）。20例患者还患有尿崩症。对于单灶性SS-LCH患者，建议在活检/手术后采取“等待观察”策略。全身治疗的情况下，SS-LCH涉及“特殊部位”或多灶性疾病，并在MS-LCH的情况下提出。整个队列的EFS和OS在5年时分别为62.2%和100%，在10年时分别为52.5%和97.6%。3例患者因给予的多种治疗引起的损伤而死亡。疾病再激活率很高（影响40%的病例），尽管进行了多线治疗，但多年来仍有几次再激活。虽然成人和儿童LCH的临床病史可能不同，但在缺乏特定的、定制的方案的情况下，成人病例的临床方法可能借鉴儿科经验。局限性疾病患者预后良好，无需任何系统治疗。应记住，通常用于儿科环境的全身治疗在成人中的潜在毒性更大。
Langerhans cell histiocytosis is rare in adults, and most of what we know about its diagnosis and treatment comes from pediatric studies. We report clinical findings and results of treatment in a retrospective series of 63 consecutive adult patients (18–76 years old), treated at our pediatric unit from 1990 to 2020 using the same approach as for children. Patients were classified as having single-system disease (SS-LCH) in 41 cases, which was unifocal in 34 of them and multifocal in 7, or multisystem disease (MS-LCH) in 17 and primary pulmonary (pLCH) in 5. Twenty patients also had diabetes insipidus. A “wait and see” strategy was recommended after biopsy/surgery for patients with unifocal SS-LCH. Systemic treatment was proposed for cases of SS-LCH involving “special sites” or with multifocal disease, and in cases of MS-LCH. EFS and OS for the cohort as a whole were 62.2% and 100%, respectively, at 5 years and 52.5% and 97.6% at 10 years. Three patients died due to the damage caused by the multiple therapies administered. The rate of disease reactivation was high (affecting 40% of cases), with several reactivations over the years despite multiple lines of treatment. Though clinical history of LCH may differ between adults and children, in the absence of specific, tailored protocols, clinical approach to adult cases may draw on pediatric experience. Patients with limited disease have a good prognosis without any need for systemic therapy. Potentially greater toxicity in adults of systemic treatments generally used in pediatric setting should be borne in mind.
DOI：
10.1016/j.hoc.2015.06.003
发表时间：
2015-10
期刊：
Hematology/oncology clinics of North America
影响因子：
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作者：
Collin M;Bigley V;McClain KL;Allen CE
通讯作者：
Allen CE