郎格罕斯细胞组织细胞增多症临床特点和诊治进展

目的　结合目前郎格罕斯细胞组织细胞增多症(LCH)研究进展,探讨LCH的临床特点、诊断、治疗、复发以及预后。方法　回顾性分析2 4年收治的LCH 5 5例,年龄2～6 7岁,男4 7例,女8例。单器官系统受侵4 0例,多器官系统受侵15例。治疗以手术切除和放疗为主,手术为部分或全部切除病灶,放疗为受累野照射(中位剂量30Gy)。结果　LCH以头颈部软组织受侵多见(占6 3.6 % ) ,骨次之(占2 3.6 % )。骨受侵率在≤15岁组和>15岁组分别为6 6 .7%和11.6 % (P <0 .0 1)。5、10年总生存率均为10 0 % ,无病生存率分别为70 .9%、5 8.4 %。5年无病生存率在≤15岁组和>15岁组分别为5 8.3%和74 .4 % (P =0 .830 ) ,单器官系统受侵组和多器官系统受侵组分别为75 .0 %和6 0 .0 % (P=0 .130 )。总复发率为4 3.6 % ,2 / 3在5年内复发,其中新病灶占75 %。结论　LCH并非儿童特发;头颈部为LCH好发部位;其骨受侵率与年龄相关;复发率较高;但无危险器官受侵的预后良好     

成年人骨朗格汉斯组织细胞增生症一例并文献复习

目的 探讨成年人骨朗格汉斯组织细胞增生症（LCH）的临床特点、治疗和预后,进一步提高对成年人骨LCH的认识.方法 回顾性分析1例成年骨LCH患者的临床资料并复习相关文献.结果 1例多发骨破坏患者经病理活组织检查证实为成年人骨LCH,排除转移癌及骨髓瘤,分型为单系统多部位,需要全身治疗.结论 成年人骨LCH罕见,临床表现多样,易误诊、漏诊,需病理证实,确诊后要进行病情评估及分型,再进行个体化治疗.     

儿童骨嗜酸性肉芽肿的临床特征、治疗及预后（附33例）

目的:探讨儿童骨嗜酸性肉芽肿(eosinophilic granuloma,EG)的临床特征、治疗和预后。方法:回顾性分析2004年01月至2021年06月我院小儿骨科33例骨EG患者,分为单病灶受累和多病灶受累两组,收集患者的一般资料、临床特征、治疗和转归情况,采用Kaplan-Meier方法估计全组及两组的无事件生存率,并用对数秩检验（log-rank检验）进行两组比较。结果:男性25例(76%),女性8例(24%),诊断时的中位年龄2.1岁(0.2岁~7岁)。主诉多为骨局部肿块、疼痛、功能受限。其中四肢受累最多,其次为脊柱、骨盆、颅骨。单病灶组21例,其中12例行局部病灶切除手术治疗,有或没有使用局部类固醇;7例行手术及术后全身化疗,有或没有使用局部类固醇;2例行活检后全身化疗。多病灶组3例行手术联合全身化疗,9例行活检后全身化疗。单病灶组1例复发,多病灶组5例进展,两组均无患儿死亡。单病灶组较多病灶组EFS高,差异有统计学意义(P=0.009),中位随访时间7.3年(0.8年~17.5年)。结论:骨EG风险较低,无明显生命威胁,预后较好。单病灶受累较多病灶受累者的复发或进展较少、手术效果好、治愈率高;多病灶受累者化疗效果较好,进行积极的治疗并坚持长期随访可减少并发症的产生,提高患者的生存质量。     

95例伴气管食管沟淋巴结转移食管癌三维放疗±化疗疗效分析

目的 探讨食管癌气管食管沟淋巴结(TGLN)转移患者3DCRT±化疗疗效。方法 对2003—2010年间符合入组条件的95例有TGLN转移的食管癌患者治疗情况进行回顾分析,分析疗效、预后影响因素及治疗失败模式等。 95例放疗 54~68 Gy分27~34次5~7周完成, 38例加“顺铂”为基础方案化疗,其中同期放化疗14例、序贯放化疗24例。Kaplan Meier 法计算生存率, Cox 模型多因素预后分析。结果 随访率100%。治疗结束后患者食管病变总有效率为94%;转移淋巴结病变治疗后有效率为100%。1、2、 3年OS分别为53%、32%、24%,中位生存时间13个月。多因素分析结果显示患者治疗前有无胸背部疼痛(P=0.041)、病变造影长度(P=0.002)及食管病变近期疗效(P=0.000)为预后影响因素。全组患者出现单纯食管复发18例,单纯淋巴结转移或复发 4例,食管复发伴淋巴结转移或复发 5例,单纯远处转移 28例,食管复发伴远处转移4例,远处转移伴淋巴结转移或复发3例,食管复发合并淋巴结转移或复发并伴远处转移 2例。结论 食管癌伴TGLN转移患者接受3DCRT ±化疗较为安全,但疗效有待进一步提高。影响食管癌伴TGLN转移患者的主要预后因素与食管病变相关。     

郎格罕组织细胞增生症26例临床分析

目的:郎格罕组织细胞增生症( Langerhans cell histiocytosis, LCH),临床表现复杂多样,容易误诊.本研究在于了解其临床特征,提高对该病的认识,减少误诊率.方法:回顾性分析本院2003年1月-2007年7月确诊的26例LCH患者的临床表现、影像、病理资料及治疗方法.结果:LCH侵犯的组织器官有骨骼20例(76.92%)、淋巴结2例(7.69%)、肺、脾、耳、肌肉各2例,肝、胃、垂体、鼻腔、口腔各1例;侵犯1个器官23例(88.46%),侵犯2个器官1例(3.85%),侵犯4个器官1例(3.85%),侵犯6个器官1例(3.85%).临床表现多样化,症状与影像学缺乏特异性表现,确诊依靠病理诊断.单发病灶以手术、放疗为主.局部治疗效果好.结论:LCH临床表现多样,误诊率高.多系统病变采取化疗,单发病灶者手术及放疗疗效好.     

基于全身化疗基础上胸部三维放疗对Ⅳ期非小细胞肺癌预后影响

目的:探讨基于全身化疗的基础上胸部原发病灶三维放疗在Ⅳ期非小细胞肺癌综合治疗中的作用.方法:2003年1月至2010年7月93例Ⅳ期NSCLC患者接受至少4周期化疗并同期胸部原发病灶三维放疗≥40Gy,对疗效及预后因素进行分析,Kaplan-Meier法计算生存率并行Logrank检验,Cox回归模型行多因素预后分析.结果:末次随访日期2013年3月,中位随访14.0个月(4.0个月～80.0个月);截止末次随访有5例患者存活,生存时间34.0个月～ 80.0个月,中位生存时间64.0个月.全组中位生存期为14.0个月(95％ CI,11.44-16.60),1年、2年、3年生存率分别为54.8％、20.4％和12.9％.61例知道确切死亡原因,48例患者死于远处转移,7例死亡时局部复发伴有远处转移,仅2例死亡时单纯局部复发.胸部原发灶放疗处方剂量≥63 Gy和＜63Gy的中位生存期分别为15.0个月(95％ CI,12.86～17.14)和8.0个月(95％ CI,5.89-10.11)(x2=10.416,P=0.001);单器官转移患者生存期比多器官转移患者长,MST为15.0个月(95％ CI,12.25-17.75)比10.0个月(95％ CI,6.04-13.96) (x2＝9.436,P =0.002).亚组分析,单器官转移患者DTpTv≥63 Gy仍是影响生存的因素(x2＝4.733,P＝0.030);多器官转移患者DTpTv≥63Gy有延长总生存的趋势(x2=3.488,P=0.062).多因素分析显示:胸部原发肿瘤(DTpTv)≥63Gy和单器官转移是影响总生存期的独立预后因素.结论:全身系统化疗的基础上,胸部原发病灶积极的放射治疗(≥63Gy)能使患者生存获益,单器官转移患者从该治疗模式中获益较大,多器官转移患者也有生存获益的趋势,积极的放射治疗可能在改善生存方面具有重要作用.     

原发皮下脂膜炎样T细胞淋巴瘤的临床表现治疗及预后

目的 探讨皮下脂膜炎样T细胞淋巴瘤(SCPTCL)的临床表现、治疗特点及预后.方法 对1999年1月至2009年1月间收治的10例SCPTCL患者的临床资料进行总结和分析.结果 患者中男性4例,女性6例,中位发病年龄为50.5岁(10～58岁).其中CD56阴性7例,阳性2例,不明1例;病变多位于躯干和四肢,有4例患者诊断前反复出现可自愈的皮下结节.多发性皮肤损害7例,单发性3例;5例伴有疼痛,3例发生溃疡.4例有内脏或淋巴结侵犯,5例伴有肝功能损伤,1例患者出现嗜血综合征.3例单发性患者采用外科切除+化疗或化放疗,其中1例失访,2例仍无病生存.7例多发性皮肤损害或伴有淋巴结、内脏受侵的患者中,1例仅行局部切除治疗,目前仍无病生存;6例采用化疗或以化疗为主的综合治疗者,其中3例为无进展生存,1例复发后采用组蛋白去乙酰化酶抑制剂治疗获得部分缓解,2例死亡.中位随访44个月(14～99个月)时,全组患者的无进展生存率(PFS)为66.7%(6/9例),总生存率(OS)为77.8%(7/9例).结论 SCPTCL病程相对惰性,皮损可自愈或反复出现.单发病变患者经综合治疗后,可获得长期无病生存;病变广泛或有皮肤外受侵者对化疗有效,但缓解时间较短,组蛋白去乙酰化酶抑制剂可用于SCPTCL的复发后治疗.     

胰腺转移性肾透明细胞癌的临床病理特征和预后分析

目的探讨胰腺转移性肾透明细胞癌患者的临床病理特征和预后。方法回顾性分析2000年1月至2018年12月在北京协和医院收治的经病理确诊的18例胰腺转移性肾透明细胞癌患者的临床病理资料。结果18例患者中,男11例,女7例;确诊肾透明细胞癌的平均年龄为51.4岁。其中左肾8例(44.4%),右肾10例(55.6%);同时性转移3例,异时性转移15例,从确诊肾透明细胞癌到发现胰腺转移的中位发生转移的时间为156个月,主要临床表现有腹痛、黄疸、消化道出血、恶心、乏力、体重下降等。其中胰腺单病灶患者7例(38.9%),多病灶患者11例(66.1%),9例患者(50.0%)除胰腺外还同时存在其他部位转移。5例患者行胰腺转移灶切除,15例患者服用靶向药物治疗。随访时间1~361.5个月,平均随访时间为171.7个月,死亡5例,生存13例,中位生存时间为122个月,5年生存率为81.4%。是否为同时性转移、是否为10年后复发、纪念斯隆-凯特琳癌症中心模型预后评分以及国际转移性肾细胞癌联合数据库评分是影响胰腺转移性肾透明细胞癌患者预后的因素。结论胰腺转移性肾透明细胞癌罕见,但预后较好,尤其是10年后复发转移至胰腺的患者,行手术切除胰腺转移灶未发现明显的生存获益。     

老年复发卵巢癌患者预后影响因素的临床分析

背景与目的:老年卵巢癌患者预后差,生存率明显低于年轻患者,本研究旨在探讨老年复发卵巢癌的临床病理特点对预后的影响.方法:回顾性分析1999年3月-2009年12月在我院治疗的铂类敏感型复发卵巢癌患者共235例,其中老年组52例(年龄≥65岁),非老年组183例(年龄＜65岁),采用单因素及多因素分析对年龄、二线化疗方案、PS评分和二次减瘤术后残瘤等预后影响因素进行分析.结果:老年组进行二次减瘤术的患者较非老年组明显下降(8.7%比24.6%,P=0.001 8).行二次减瘤术患者的中位生存时间为35.6个月,而未行二次减瘤术者为20.7个月,差异有统计学意义(P=0.02).二线化疗中,老年组接受单药铂类化疗较非老年组多(45.3%比22.1%,P=0.006).二线化疗为单药铂类化疗的中位生存时间为21.6个月,而联合铂类化疗的为32.8个月,差异有统计学意义(P=0.006).中位生存期老年组为23.7个月,非老年组为31.2个月(P=0.165).多因素分析结果显示,复发年龄、分期、二线化疗方案、复发时PS评分、初次化疗后肿瘤无进展生存期、复发病灶数、手术后残瘤等因素与生存期独立相关(P＜0.05).结论:复发年龄、分期和二线化疗为铂类联合化疗、PS评分、初次化疗后肿瘤无进展生存期、复发病灶数、二次减瘤术后残瘤是复发卵巢癌预后的独立影响因素,正确地选择二次减瘤术加铂类联合化疗作为老年复发卵巢癌的二线治疗将提高疗效.     

脑原发淋巴瘤19例临床分析

目的：分析脑原发淋巴瘤的临床特点，治疗和预后情况，结合文献复习探讨疗效的改进。方法：1990年4月至2000年8月收治19例患者，中位年龄51岁，均无免疫抑制的证据。初发病灶单发者11例，多发者8例。68．4％的病变为B细胞来源，中高度恶性多见。17例手术后放射治疗，其中10例还接受化疗；2例未手术直接放射治疗，其中1例还接受化疗。结果：近期疗效良好，全组生存时间9－65个月（中位20个月），1、3、5年生存率分别为79．0％、30．4％和8．1％。单因素分析显示患者年龄对生存率有显著影响，病灶个数、放射治疗剂量、加用化疗对生存均无 显著影响。4例疗后有无复发不详，13例患者疗后出现病变复发，复发灶均位于中枢神经系统内，其中7例初发病灶未控或复发，6例诊为癌性室管膜炎。神经毒性为主要的治疗并发症。结论：脑原发淋巴瘤发病率近年明显上升，疗效不理想，60岁以上的患者预后不良。增加放射治疗剂量并不能改善肿瘤控制，综合治疗的进展有望改进疗效。     

郎格罕细胞组织细胞增生症的临床特征与诊断

背景与目的：郎格罕细胞组织细胞增生症（Langerhams cell hyperplasia,LCH）发病逐步增多,临床表现多样化而易误诊。本研究在于了解其临床特征,有利于早期诊断与治疗,评价预后。方法：将近10年来中山大学附属第一医院诊断的44例LCH作回顾性分析。结果：LCH主要发生于小儿,占72．7％（32／44）。成人占27．3％（12／44）,2岁以下小儿占小儿发病的46．9％（15／32）。侵犯的组织器官常见有骨骼20例（45．5％）,淋巴结20例（45．5％）,皮肤16例（36．4％）,肝脾16例（36．4％）,肺14例（31．8％）,骨髓6例（13．6％）,耳4例（9．1％）,眼2例（4．5％）,垂体2例,胸腺1例。其中1个脏器受损10例,2个脏器受损11例,3个脏器受损9例,4个脏器受损7例,5个以上脏器受损7例,肝、肺、骨髓等多功能受损中,小儿22例,成人4例．表现多样化。误诊26例（59．1％）,其中误诊皮肤病6例,其它血液病7例,骨骼及骨髓疾病7例、肺结核2例、尿崩症2例、耳及眼疾病各1例,小儿病例并发支气管肺炎6例,致败血症4例;死f6例,其中2岁以下小儿5例。结论：LCH临床易于误诊,2岁以下小儿多脏器功能衰竭及并发支气管肺炎常见,且死亡率高。作相关组织细胞病理检查有助鉴别,有利早期诊断,早治疗,防止严重并发症。     

以胆管炎为主要表现的朗格罕细胞组织细胞增多症临床特点分析

目的:总结朗格罕细胞组织细胞增生症(langerhans cell histiocytosis,LCH)的主要临床特点、诊断、治疗以及预后,尤其是以胆管炎为主要表现的病例.方法:回顾性分析2012年7月-2016年4月至湖南省儿童医院确诊并住院治疗的12例LCH患儿的临床表现、实验室检测、骨髓细胞学检查以及治疗情况,治疗以化疗为主.单器官受累8例,多器官系统受累4例.结果:12例LCH患儿中,11例有程度不一的黄疸,8例超声表现为肝大,2例骨骼系统有破坏,6例有肺部侵犯.结论:LCH的诊断是复杂的,可能涉及多个器官系统,其临床表现和疾病变化的过程是从早期一个孤立的表现到后来多系统表现,通过对这些病例的研究可能有助于我们对LCH的病理生理学的认识和理解,从而提高临床对LCH的诊断和治疗.     

Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society

Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year survival 93.6% (95% CI 84.7-97.4) versus 87.5% (95% CI 65.5-95.9), respectively; P value 0.1). LCH in adults is most often a multisystem disease with the highest mortality seen in patients with isolated pulmonary involvement. It should be included in the differential diagnosis of disseminated or localised disease of the bone, skin and mucosa, as well as the lung and the endocrine and central nervous system, regardless of the age of the patient. A prospective international therapeutic study is warranted.     

A NEW LOOK AT LANGERHANS CELL HISTIOCYTOSIS： REVIEW OF A SERIES OF 55 CASES

Langerhans cell histiocytosis (LCH) is characterized by the proliferation of abnormal histiocytes-Langerhans cells, which develop from bone marrow-derived dendritic cells[1,2]. The cause of the proliferation and accumulation of these cells remains unclear. The main clinical manifestations of LCH are single or multiple bone, skin, or soft-tissue lesions with or without involvement of organs at risk (liver, spleen, bone marrow, and lungs). LCH can be classified assingle-system or multisystem…     

Clinical and prognostic features of Langerhans cell histiocytosis in adults

Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal expansion of CD1a⁺CD207⁺ myeloid dendritic cells. The features of LCH are mainly described in children and remain poorly defined in adults; therefore, we conducted a nationwide survey to collect clinical data from 148 adult patients with LCH. The median age at diagnosis was 46.5 (range: 20–87) years with male predominance (60.8%). Among the 86 patients with detailed treatment information, 40 (46.5%) had single system LCH, whereas 46 (53.5%) had multisystem LCH. Moreover, 19 patients (22.1%) had an additional malignancy. BRAF V600E in plasma cell-free DNA was associated with a low overall survival (OS) rate and the risk of the pituitary gland and central nervous system involvement. At a median follow-up of 55 months from diagnosis, six patients (7.0%) had died, and the four patients with LCH-related death did not respond to initial chemotherapy. The OS probability at 5 years post-diagnosis was 90.6% (95% confidence interval: 79.8–95.8). Multivariate analysis showed that patients aged ≥60 years at diagnosis had a relatively poor prognosis. The probability of event-free survival at 5 years was 52.1% (95% confidence interval: 36.6–65.5), with 57 patients requiring chemotherapy. In this study, we first revealed the high rate of relapse after chemotherapy and mortality of poor responders in adults as well as children. Therefore, prospective therapeutic studies of adults with LCH using targeted therapies are needed to improve outcomes in adults with LCH.     

骨朗格汉斯细胞组织细胞增生症25例临床分析

 目的　分析骨朗格汉斯细胞组织细胞增生症（LCH）的临床特点,总结LCH诊断和治疗的方法。方法　回顾性分析2004年2月至2012年2月收治的25例经病理证实的LCH患者的临床资料及随访结果。结果　25例患者中男18例,女7例;中位年龄17岁。单发病灶17例,其中颅骨11例,多发病灶6例。首发症状多为疼痛和局部肿块,全身症状少见。主要症状多为局部疼痛,影像学表现为溶骨性改变,12例伴周围软组织肿胀。病理表现为分化好的组织细胞增生及大量嗜酸性粒细胞浸润,CD1a、S100、Vimentin、CD68免疫组织化学阳性率高。单发病例采用手术治疗为主,辅以放疗或化疗。多发病例以化疗为主,辅以放疗。仅累及骨的患者疗效满意,累及其他脏器的2例患者死亡。结论　骨LCH男性发病明显高于女性,好发于儿童和青少年,以单发病灶为多,颅骨侵犯多见。临床表现主要为局部疼痛和肿块,病理活组织检查是确诊的首选方法,治疗宜采用综合疗法。预后与骨病损范围和病理类型以及其他脏器受累情况,大部分病例预后好。     

Langerhans cell histiocytosis with central nervous system involvement--complete response to 2-chlorodeoxyadenosine after failure of tyrosine kinase inhibitor therapies with sorafenib and imatinib

Langerhans cell histiocytosis (LCH) is rare in adults, and only a subset of these patients suffers from central nervous system (CNS) involvement. Hence, evidence-based treatment recommendations are lacking. A case of a 20-year-old student with multisystem LCH and extensive CNS involvement is described, who showed a durable response to 2-chlorodeoxyadenosine after prior therapies with the tyrosine kinase inhibitors sorafenib and imatinib. In accordance to the experiences provided by other case series, which are reviewed herein, 2-chlorodeoxyadenosine can be considered an effective and safe option for adult LCH with CNS involvement.     

成人噬血细胞性淋巴组织细胞增生症21例临床分析

目的:探讨成人噬血细胞性淋巴组织细胞增生症(HLH)的临床特点。方法:对2005年1月－2015年1月在南京医科大学第二附属医院血液科收治的21例成人HLH患者的临床资料进行回顾性分析。结果:21例患者初治时均表现为高热、肝脾肿大、凝血功能异常、血常规三系或二系受累。病因分析中7例（33.3%）为血液肿瘤相关性;10例（47.6%）为感染,其中3例确诊为发热伴血小板减少综合征（新型布尼亚病毒核酸检测阳性）;1例（4.8%）肿瘤相关性（骨髓活检示转移癌）;3例（14.3%）原因不明。随访中死亡16例,存活3例（最长至今已5年7个月）,2例失访;生存时间为4天～5.7年（中位生存时间183天）。结论:HLH为罕见的致死性疾病,成人更为少见。临床表现复杂,常伴有多脏器受损,病情凶险,进展迅速。预后大多不良。发病机制和治疗手段有待进一步研究。     

Clinico-biologic profile of Langerhans cell histiocytosis: a single institutional study

CONTEXT: Langerhans cell histiocytosis (LCH) is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject. AIM: To present the experience of management of LCH at a single institution. SETTINGS AND DESIGN: This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002. MATERIALS AND METHODS: Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded. STATISTICAL ANALYSIS USED: Statistical analyses were done using Student's 't' test, test for proportion and survival estimates based on the Kaplan-Meier method. RESULTS: The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80%) required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae. CONCLUSIONS: The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrollment in clinical trials with innovative therapeutic strategies to improve outcome.