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。     

宫颈朗格汉斯细胞组织细胞增生症的临床病理分析

目的 探讨女性生殖道朗格汉斯细胞组织细胞增生症的临床病理学特征,以加深此病在宫颈等少见部位的认识.方法 对1例宫颈LCH进行HE及免疫组化染色,观察其临床病理特征,并复习相关文献.结果 患者女性,31岁,确诊为宫颈LCH,镜下朗格汉斯细胞弥漫增生,肿瘤细胞中等大小,细胞质淡粉染,核呈卵圆形,可见核沟,核仁不明显;背景可...     

成人朗格汉斯细胞组织细胞增生症1例

1 病例资料 患者,男,45岁,以"反复发热1月余"为主诉于2013年3月12日入我院.患者于2013年2月开始无明显诱因出现自觉发热,伴右上腹疼痛、乏力,全身酸困.口服感冒药物,症状稍好转.后上述症状反复出现,在外院反复抗感染治疗,症状无改善,体温最高38.5 ℃.查上腹部MRI提示肝脾大,腹腔积液.转入我院,门诊以"发热待查"收住院.发病以来,神志清,精神不佳,纳少,大小便可,体质量减轻约5 kg.入院查体:体温38.4 ℃,心率100次/min,呼吸频率24次/min,血压110/70 mmHg,发育正常,营养中等,躯干部皮肤可见红色丘疹,压之褪色.颈部、双侧腋窝下、腹股沟处可触及多个肿大淋巴结,最大直径约1.5 cm,可活动,压痛.双肺呼吸音清,未闻及啰音.心律齐,未闻及杂音.腹部膨隆,肝肋下三横指,脾肋下三横指可触及.双下肢无水肿.胸片提示双肺纹理增粗.上腹部MRI示:1)肝脏体积增大;2)脾大,脾脏外缘多发异常信号,考虑脾梗死可能.初步诊断:发热待查.     

骨朗格汉斯细胞组织细胞增生症3例临床病理分析北大核心CSCD

目的探讨骨朗格汉斯细胞组织细胞增生症（langerhans cell histiocytosis,LCH）的临床病理特征、免疫表型、鉴别诊断及生物学行为。方法对3例骨LcH临床特征、组织学形态和免疫组织化学结果结合相关文献进行分析。结果2例男性,1例女性,发病年龄7～12岁（平均8．7岁）。病变部位分别位于肱骨、胫骨及额骨;镜下示Langerhans胞异常增生,伴有数量不等的嗜酸性粒细胞、淋巴细胞、中性白细胞及多核巨细胞;免疫组织化学提示,3例Langerhans细胞弥漫表达CD1a和s100。结论绝大多数发生于骨朗格汉斯细胞组织细胞增生症患者预后较好。其预后与病变部位、累及范围、有无器官功能异常及年龄相关。     

肱骨朗格汉斯细胞组织细胞增生症二例并文献复习

朗格汉斯细胞组织细胞增生症（1angerhanscellhistiocytosis,LCH）是一种少见的疾病,曾被称为组织细胞增生症X,可以累及全身多种脏器,包括肺、肝脏、皮肤、黏膜组织以及淋巴结,好发于儿童和年轻人,临床表现多样,可表现为单灶性病变或多系统病变,其病因及发病机制尚未明确,目前多数研究认为是肿瘤性病变【1】,也有学者认为是免疫调节异常所致的疾病【2】。发生于肱骨的LCH则更为少见,我院手术治疗肱骨的朗格汉斯细胞组织细胞增生症2例,结合文献分析如下。     

成人朗格汉斯细胞组织细胞增生症漏诊1例报告并文献复习

目的:分析朗格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis,LCH）临床特点及漏诊原因,以期提高对该病的认识,降低漏诊率。方法:回顾分析1例LCH患者的临床特征、诊断、治疗,并结合国内外文献报道,分析漏诊原因。结果:患者男性,27岁,以双侧下颌骨肿物并胸部不适为首发表现。于5月行口腔病理活检,活检结果提示,免疫表型:CD1a（-）、CD207（-）、S-100（±）,漏诊LCH,未予LCH对症处理和治疗;后于8月行纤支镜下冷冻肺活检术,免疫表型:CD1a（+）、CD207（+）、S-100（+）,明确诊断LCH。结论:口腔颌骨组织病变伴有肺或全身其他部位骨组织的病变,临床上应考虑LCH的可能,需尽快行免疫组化检查,为避免漏诊应采取合适的活检取材方法。LCH确诊后应完善相关全身检查以避免存在其他靶器官漏诊,并基于全身脏器受累情况,采用合理治疗方案以获得更好的疗效和预后。     

成人股骨朗格汉斯细胞组织细胞增生症一例报告

朗格汉斯细胞组织细胞增生症(langerhanscell histiocytosis,LCH)是一组罕见的属于单核细胞系统的特定树突细胞和网状细胞增生为共同特点的疾病,成年病例罕见[1].由于其缺乏典型的临床病史和特异性的临床表现常常导致漏诊和误诊.近期我们遇原发于成人股骨LCH 1例,现报告如下.     

骨朗格汉斯组织细胞增生症诊断难点分析

  目的  分析讨论骨朗格汉斯组织细胞增生症（bone Langerhans cell histiocytosis,BLCH）诊断中的难点及应对策略。  方法  回顾性分析上海市第一人民医院2014年1月至2020年12月期间诊断为B-LCH患者的临床、影像及病理资料,对诊断病例和未诊断病例进行逐例分析讨论。  结果  1）临床诊断率为7%（2/30）,难点在于缺乏诊断意识及临床特征特异性不足。患者年龄<14岁;好发于颅骨、脊柱等骨组织;局部疼痛,疾病进展相对缓慢;累及其他系统,如累及垂体引起尿崩症等,以及复发病例对诊断有提示意义。2）影像诊断率为27%（8/30）,难点在于影像表现的多样性导致该病与感染、偏良性病变、恶性病变均容易混淆。诊断时需仔细甄别影像特征,并密切结合临床综合分析,进一步行CT三维重建、MRI、PET-CT检查可提高该病的诊断率。3）病理初次、再次、三次诊断率分别为60%、33%、7%,难点在于当穿刺标本量少、大量炎症背景、肿瘤细胞特征不典型时会造成诊断困难。病理医生应多阅片,多结合临床,加强对该病的认识和诊断能力,当临床-影像有指向性意见时,可降低病理诊断难度和降低潜在的误诊、漏诊风险。  结论  B-LCH是一种发生于骨的罕见造血系统组织细胞类肿瘤,临床、影像、病理诊断均有难度,各科诊断医生需提高对该病的认识和诊断意识,采用临床-影像-病理等多学科联合诊疗方法对提高诊断准确率尤其重要。      

成年人朗格汉斯细胞组织细胞增生症21例临床病理特征分析

目的 分析成年人朗格汉斯细胞组织细胞增生症(LCH)的临床特征、治疗及预后.方法 对2010年3月至2017年3月南京医科大学第一附属医院及南京市第二医院收治的年龄≥18岁的21例成年LCH患者资料进行回顾性分析,总结其临床表现、实验室检查结果 、治疗方法和预后.结果21例LCH患者中男性14例,女性7例,中位年龄43岁(22~62岁).4例为单系统、单病灶,5例为单系统、多病灶(Hand-Schuller-Christian病),12例为多系统、多病灶(Letter-Siwe病);其中7例有重要器官受累(肺部3例、骨髓2例、肝脏1例、脾脏1例),为高风险患者.所有患者中位生存时间为36个月(1~89个月),其中9例单系统病变及5例多系统病变伴低风险患者均生存,病情稳定,未出现疾病复发,生存时间为4~89个月;7例多系统病变伴高风险患者中3例生存,病情稳定,未复发,4例死亡.结论 成年人LCH发病率较低,患者中男性居多,与儿童LCH不同,成年人LCH临床多为多病灶、多系统病变.多系统病变伴高风险患者治疗反应差,需探索新型治疗方案.     

朗格汉斯细胞组织细胞增生症

朗格汉斯（Langerhans）细胞组织细胞增生症,以往曾称为组织细胞增生症X,根据临床表现可分为嗜酸性肉芽肿、Hand—Sch ǖ ller—Christian病、Letterer—Siwe三个亚型。本病发病率估计1／20万～1／200万,主要发生在婴儿和儿童。     

17例伴有肺部改变的朗格汉斯细胞组织细胞增生症的临床观察

目的 探讨儿童伴肺部改变的朗格汉斯细胞组织细胞增生症（PLCH）的临床表现、CT特征性表现、诊断及治疗特点,并分析其与预后的关系,为PLCH患者临床方案的制定提供依据。方法 回顾性分析本院2008年1月至2014年1月经病理确诊的17例PLCH患儿的临床特征、影像学资料及接受Ⅲ型化疗方案（诱导阶段6周+维持阶段52周）化疗后的疗效和预后。结果 17例PLCH患儿起病时伴有肺部弥漫性改变为主的多脏器损害,在疾病发展过程中,胸部CT表现出特征性变化。17例均接受Ⅲ型方案化疗,其中8例完全缓解,预后良好;4例化疗后复发,其中1例预后良好,3例死亡;3例化疗中疾病进展放弃治疗,死亡;1例化疗中疾病进展再诱导化疗,目前持续化疗中,存活;1例化疗维持治疗中,目前持续随访。结论 儿童伴肺部改变的LCH多为婴幼儿起病,伴多脏器损害,早期高强度化疗可改善预后,但部分病例肺部进展明显,预后不良。     

A case of isolated Langerhans cell histiocytosis of the hypothalamus with remission and regrowth after surgery

We describe a new case of isolated Langerhans cell histiocytosis (LCH) of the hypothalamus. A 53-year-old female patient presented with polydipsia, headache, anorexia, and fatigue. Neurological imaging revealed a mass projecting from the hypothalamus into the third ventricle. Gross total removal of the tumor was performed. Light microscopy showed LCH, and immunohistochemical studies revealed S-100 and CD1a immunoreactivity in the Langerhans cells. Although the most common CNS site for LCH is the hypothalamus, isolated hypothalamic LCH, confirmed by biopsy, is very rare. The residual mass appeared to remit spontaneously 3.5 years after surgery, with regrowth 3 years later.     

A case of hypersensitivity syndrome resembling Langerhans cell histiocytosis during phenobarbital prophylaxis for convulsion.

The case of a two-year-old girl with generalized histiocytosis, probably induced by phenobarbital, is reported. Symptoms, including intermittent fever, systemic lymphadenopathy, maculopapular skin eruption and hepatosplenomegaly, suggested Langerhans cell histiocytosis. Laboratory examinations revealed leukocytosis with lymphocytosis and eosinophilia and a high LDH serum level, while GOT and GPT were within normal ranges. Cytological studies of lymph node and pleural effusion specimens revealed proliferation and infiltration of Langerhans cell histiocytes with eosinophilia. No histiocyte proliferation was observed in the bone marrow or skin. The clinical manifestations shown by the patient were, however, transient, and improved spontaneously after the discontinuation of phenobarbital. The case was considered to be one of phenobarbital hypersensitivity syndrome based on clinical course and laboratory findings. The mechanism and differential diagnosis of the syndrome are discussed.     

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…     

Langerhans cell histiocytosis: Current concepts and treatments

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of cells with the phenotype of activated Langerhans cells. The diagnosis of LCH is often delayed or missed. Many questions about LCH remain to be answered, including whether it is caused by a malignancy or by immune dysregulation. Data from the early 1990s showed that LCH consisted of an accumulation of monoclonal LCH cells, suggesting a neoplastic disorder. However, further investigations with current sophisticated techniques have not shown consistent genomic aberrations. Recent data which suggests a role for an IL-17A dependant pathway of dendritic cell fusion in LCH remains to be proven. The most recent data taken together swing the pendulum towards an immunologic aberration.     

Solitary Langerhans cell histiocytosis of frontal lobe: a case report and literature review

The brain parenchymal Langerhans cell histiocytosis （LCH） without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of consciousness 20 days prior to admission, and recurrent seizure 2 weeks later. Brain magnetic resonance imaging （MRI） showed an irregularly mass with enhancement involving the right frontal lobe. Microscopically, the lesion was characterized by sheets of Langerhans cells in addition to reactive inflammatory elements. Immunohistochemically, Langerhans cells were positive for Langerin, CDla and S-100 protein. The patient received no chemotherapy or radiotherapy after surgery. After 24 months of follow-up, no recurrence or other systemic lesions were observed. Although there is no standard treatment for solitary cerebral LCH, the prognosis generally appears to be good.     

Fine-needle cytology of cutaneous juvenile xanthogranuloma and langerhans cell histiocytosis

BACKGROUND:

In pediatric patients, a cutaneous nodule is usually diagnosed by performing an excisional biopsy, but fine‐needle cytology (FNC) is a safer and noninvasive diagnostic method widely used to obtain diagnostic specimens with little stress to the patient. The authors compared the ability of FNC and biopsy to differentiate Langerhans cell histiocytosis (LCH) from juvenile xanthogranuloma (JXG).

METHODS:

Correlating cytological results with histological findings, the authors reviewed 27 patients (15 males and 12 females; mean age, 37 months; range, 1 month to 14 years) admitted to the University of Padua Department of Pediatrics from 1998 to 2010.

RESULTS:

Cytology smears were adequate in all 27 (100%) patients: 14 (52%) were classified as having JXG, 12 (44%) as having LCH, and 1 (4%) as having a doubtful finding. A biopsy was also performed in 20 of these patients, and in all but 1, the 2 methods were completely concordant.

CONCLUSIONS:

FNC is safe and useful in the diagnostic workup of pediatric patients with cutaneous nodules and has no contraindications to its use as the initial diagnostic procedure. Cancer (Cancer Cytopathol) 2011;. © 2010 American Cancer Society.     

MALIGNANT HISTIOCYTOSIS: A STUDY ON CLINICOPATHOLOGICAL FEATURES AND CELL ORIGIN

Thirty- one autopsy cases previously diagnosed as malignant histiocytosis (MH) were studied by means of immunohistochemical staining. Antibodies detecting the formalin resistant epitopes on T- cells, B- cells and those of histiocyte/monocyte origin were used. It was shown that the malignant histiocytes reacted only to the cell markers derived from histlocyte/monocyte. and only a part of lymphocytes showed positive reaction to the T and B cell markers. It is suggested that the histiocyte/monocyte lineage is the possible origin of the malignant proliferating cells in MH. The clinicopathological features and the differentiation of MH from familial erythrophagocytic lymphohistiocytosis, virus-associated hemophagocytic syndrome and malignant lymphoma are described. The pathogenesis. the causes of death and the points for attention in the treatment of MH are also discussed.     

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.