Recurrent multisystem Langerhans cell histiocytosis involving the female genitalia: A case report

BACKGROUND Langerhans cell histiocytosis(LCH)is a histiocytic proliferative disease caused by clonal proliferation of Langerhans cells,which is currently defined as an inflam-matory myeloid tumor.It is rare in adults,with an incidence of 1–2 per million,and is highly heterogeneous in clinical presentation,with unpredictable disease progression and outcome.CASE SUMMARY A 52-year-old postmenopausal female patient presented to the gynecology department in July 2023 with bilateral vulvar masses.She was diagnosed with recurrent multisystem LCH.The patient had previously been diagnosed with a single-system and single-focal LCH in October 2021 due to a right maxillofacial mass,which resolved after surgical treatment.A chemotherapy regimen was developed after multidisciplinary consultation.Six cycles of chemotherapy resulted in partial remission,and maintenance chemotherapy is currently being administered.CONCLUSION Recurrent LCH involving the bilateral vulva has been poorly reported.Compre-hensive imaging and pathological evaluation is important for diagnosis.The model of joint multidisciplinary specialist diagnosis and treatment is worthy of clinical application.

Multisystemic recurrent Langerhans cell histiocytosis misdiagnosed with chronic inflammation at the first diagnosis:A case report

BACKGROUND Langerhans cell histiocytosis(LCH)is characterized by diabetes insipidus and is an uncommon occurrence.Pathological biopsies still have a certain degree of diagnostic probability.We present a case in which LCH initially affected the pituitary gland.This resulted in a misdiagnosis of chronic inflammation upon pathological examination.CASE SUMMARY A 25-year-old female exhibited symptoms of diabetes insipidus.Magnetic resonance imaging revealed an enhanced foci in the pituitary gland.After surgical resection of the pituitary lesion,the pathological diagnosis was chronic inflam-mation.However,the patient later experienced bone destruction in the skull and lower limb bones.After the lower limb bone lesion was compared with the initial pituitary lesion,the final diagnosis was modified to LCH.The patient was treated with multiple chemotherapy courses.However,the patient’s condition gradually worsened,and she eventually passed away at home.CONCLUSION LCH should be considered when patients exhibit diabetes insipidus and absence of high signal intensity in the pituitary gland on sagittal T1-weighted image and abnormal enhancement in the pituitary region.

Langerhans cell histiocytosis misdiagnosed as thyroid malignancy: A case report

BACKGROUND The incidence of Langerhans cell histiocytosis(LCH) is low, and involvement of the thyroid is even rarer, which results in high missed diagnosis or misdiagnosis rates.CASE SUMMARY We report a young woman with a thyroid nodule. Thyroid malignancy was suggested by fine needle aspiration, but she was eventually diagnosed with multisystem LCH, thus avoiding thyroidectomy.CONCLUSION The clinical manifestations of LCH involving the thyroid are atypical, and the diagnosis depends on pathology. Surgery is the main method for treating primary thyroid LCH, while chemotherapy is the main treatment method for multisystem LCH.

Adult localized Langerhans cell histiocytosis:A case report

BACKGROUND Langerhans cell histiocytosis(LCH)is a rare clonal proliferative disease of Langerhans cells with unknown pathogenesis.An increasing number of clinicians recognize that LCH has a wide clinical spectrum and a highly varied course.Adults rarely develop LCH.Here,we report a case of adult localized LCH.CASE SUMMARY A 32-year-old woman presented with plaques and ulcers on the vulva and crissum,accompanied by pain that persisted for more than one year.Physical examination revealed a red-infiltrating plaque with ulcerations and exudates in the vulva and crissum.Pathological examination revealed a diffuse infiltration of lymphocytes,eosinophilic granulocytes,and histiocytoid cells in the superficial dermis.Proliferative histiocytoid cells showed mild atypia,partly with kidneyshaped nuclei.Immunohistochemical examination showed that the histiocytoid cells were positive for S100 protein and CD1 and weakly positive for CD68(20%+),with a Ki-67 index of 30%.Laboratory tests did not reveal any other systemic damage.The patient was diagnosed with adult localized LCH and was prescribed oral prednisone(20 mg)once daily.The skin lesions gradually improved and are still being followed-up.CONCLUSION Adult localized LCH is rare and must be differentiated from other common conditions.

A Rare Presentation of Erdheim-Chester Disease Overlaped with Langerhans Histiocytosis

Erdheim-Chester Disease (ECD) is a rare condition and has various differential diagnoses with other forms of histiocytosis, classified as one of non-Langerhans histiocytosis. The diagnosis of this condition remains challenging because its presentation includes non-specific systemic manifestations that can affect different organs caused by deposition of lipids and fibrosis. Most common include bone pain followed by progressive weakness and different lung manifestations. This case is about a rare presentation of ECD with Langerhans Histiocytosis as overlap syndrome, with findings of both diseases in a middle aged woman that presented dyspnea as the first symptom. The patient was treated initially as heart failure and remained without any improvement, being admitted to investigate. After a stricted follow-up, bone and lung involvement were noticed and a skin biopsy unveiled xanthomatized macrophages accompanied by Touton giant cells. This condition remains an important clinical entity and should provide new insights for clinicians dealing with respiratory diseases.

淋巴结Langerhans细胞组织细胞增生症临床病理及免疫组化研究

目的：研究以淋巴结首发的ＬＣＨ形态结构特征、免疫表型、临床表现及鉴别诊断。方法：应用ＨＥ染色和免疫组化ＡＢＣ法标记Ｏ１０和Ｓ－１００阳性以确定诊断。结果：１４例淋巴结ＬＣＨ有４种组合类型：（１）肾形及卵圆形细胞４例；（２）咖啡豆样细胞３例；（３）咖啡豆样及大圆形细胞５例；（４）大圆形细胞２例。结论：咖啡豆样细胞是ＬＣＨ的特征细胞，具有重要诊断价值，嗜酸性粒细胞是ＬＣＨ重要的组成成分之一。根据细胞形态、分化程度和不同组合，ＬＣＨ分为：①反应性，②交界性，③低度恶性，④高度恶性（肉瘤型）。

高梯度磁场免疫磁珠法分离人表皮Langerhans细胞

采用鼠抗人 OKT6单克隆抗体与包被羊抗鼠 Ig G免疫磁珠的特异性结合 ,利用钢毛产生高梯度磁场 ,进行免疫磁珠法分离纯化人表皮 L C。结果表明 LC纯化率超过 99% ,细胞活性超过 95%。该方法简单、快速、价廉、纯化率高 ,不受样本量的限制 ,易在无菌下分离纯化 。

宫颈癌浸润淋巴细胞和Langerhans细胞免疫组织化学研究

目的：研究宫颈癌浸润淋巴细胞（ＴｉＬ）、Ｌａｎｇｅｒｈａｎｓ细胞（ＬＣ）分布及与淋巴细胞活性标记物ＨＬＡ－ＤＲ和转铁蛋白受体（ＴｆＲ）的关系。方法：应用ＣＤ４，ＣＤ８，ＯＫＴ１１，ＯＫＴ９，ＨＬＡ－ＤＲ和Ｓ－１００抗体及ＡＢＣ免疫组化法。结果：ＣＤ４，ＣＤ８，ＯＫＴ１１阳性细胞随肿瘤发展而增多，并和转铁蛋白受体（ＴｆＲ）及ＨＬＡ－ＤＲ阳性表达密切关联。ＬＣ细胞数目随肿瘤分期增高和ＴｉＬ密度增加而增多。结论：ＴｉＬ和ＬＣ在宫颈癌中数量增多和活性增高的作用尚不清楚，它可能是宫颈癌局部的免疫反应表现。

黄芪增免散对围手术期食管癌组织Langerhans细胞的影响

目的观察黄芪增免散对围手术期食管癌组织Langerhans细胞(LC)的影响。方法采用黄芪增免散治疗围手术期食管癌患者36例,观察手术切除食管组织内LC的变化及形态特征。结果与对照组比较,服药组食管癌组织内LC显著增多(P<0.05),且与癌细胞密切接触,反映对癌细胞杀伤作用增强。随着肿瘤分化程度降低,浸润深度增加,LC及间质淋巴细胞数量明显减少(P<0.01),显示肿瘤对机体免疫功能的损害。结论围手术期食管癌患者免疫调节治疗可改善细胞功能。

食管癌发生过程中Langerhans细胞立体结构的观察

目的 :观察食管癌发生过程中Langerhans细胞 (Langerhanscell,LC)的三维立体结构变化。方法 :采用免疫荧光技术对 2 2例正常食管粘膜、2 0例癌旁组织和 2 3例癌组织中LC进行标记 ,通过激光共聚焦扫描显微镜对LC进行断层扫描和三维立体结构重建。结果 :断层扫描和图象分析显示正常食管粘膜中LC相对面积大 ,形状因子相对值小。癌旁粘膜和癌组织中LC相比面积明显缩小 ,形状因子相对值大 ,正常食管粘膜、癌旁组织和癌组织中LC数量减少 ,胞浆突起逐渐减少 ,立体结构趋向圆形。结论 :食管癌发生过程中LC体积缩小 ,数量减少 ,胞浆突起逐渐减少 ,提示其抗原递呈的功能减退 。

Langerhans细胞与瘢痕疙瘩形成的相关性

目的探讨Langerhans细胞与瘢痕疙瘩形成的相关性。方法应用免疫组织化学(ABC法)观察S-100蛋白及CD1a在瘢痕疙瘩及增生性瘢痕与正常皮肤组织中的表达。结果瘢痕疙瘩及增生性瘢痕组织中S-100蛋白及CD1a免疫反应阳性的Langer-hans细胞明显增多。瘢痕疙瘩及增生性瘢痕与正常皮肤之间差异非常显著(P<0.01)。瘢痕疙瘩与增生性瘢痕之间差异不显著(P>0.05)。结论Langerhans细胞与瘢痕疙瘩的形成关系密切。

瘢痕疙瘩中Langerhans细胞的电镜观察

目的:观察瘢痕疙瘩组织中Langerhans细胞的超微结构,探讨其与瘢痕疙瘩形成的关系。方法:应用电镜技术观察瘢痕疙瘩及增生性瘢痕切除标本各6例及4例正常皮肤组织中Langerhans细胞的形态,观察其分布特征。结果:电镜下表皮细胞层中朗格罕细胞核形多不规则,胞质中等电子密度,有较多线粒体和溶酶体,扁平囊泡较多。结论:Langerhans细胞在瘢痕疙瘩组织中功能活跃,Langerhans细胞与瘢痕疙瘩的形成关系密切。

舌癌中Langerhans细胞的图像分析研究

目的 :探讨Langerhans细胞学与舌癌发生、发展的关系。方法 :采用电子计算机图像分析方法对 30例人类舌癌标本中正常上皮、不典型增生、原位癌及浸润癌ATP酶阳性的Langerhans细胞进行了数量及形态定量研究。结果 :研究发现各期中Langerhans细胞的数量不同 ,不典型增生中最少 (P <0 .0 5 ) ,早期浸润癌数量明显增多 (P <0 .0 5 )。图像分析发现病变各期Langerhans细胞的面密度、体密度、形态因子均较正常上皮中有明显改变 (P <0 .0 5 ) ,但与表面基本相同 (P >0 .0 5 )。结论 :Langerhans细胞数量和形态改变可能与人类舌癌的发生、发展有关。

食管鳞癌组织中Langerhans细胞水平及意义

采用免疫组化SP法检测39例正常食管上皮、23例不典型增生及39例食管鳞癌组织中S-100蛋白的表达,以观察其中Langerhans细胞(LC)的水平。结果正常食管上皮、癌旁不典型增生和鳞癌组织中LC阳性率分别为64.10%、30.43%、41.03%,P<0.05。高、中、低分化鳞癌中LC阳性率分别为77.78%、45.45%、21.05%,P<0.01;侵犯浅肌层、深肌层和纤维膜的鳞癌中LC阳性率分别为33.33%、40.00%、42.86%,P<0.05;无和有淋巴转移的鳞癌中LC阳性率分别为53.85%、15.38%,P<0.01。认为鳞癌中LC数量减少;LC数量减少程度与食管鳞癌的分化程度及是否发生淋巴转移有关,而与浸润深度无关。

病理性瘢痕中Langerhans细胞的形态学研究

目的:探讨Langerhans细胞与病理性瘢痕的相关性。方法:应用免疫组织化学法(ABC法)观察瘢痕疙瘩、增生性瘢痕及正常皮肤组织中S-100蛋白和CD1a的表达,用透射电镜观察这三种组织中的Langerhans细胞的超微结构。结果:瘢痕疙瘩及增生性瘢痕组织中对S-100蛋白和CD1a免疫反应阳性的Langerhans细胞较正常皮肤中明显增多。瘢痕疙瘩及增生性瘢痕与正常皮肤之间有显著性差异(P<0.05)。瘢痕疙瘩与增生性瘢痕之间差异不显著(P>0.05)。电镜下表皮细胞层中Langerhans细胞核形多不规则,胞质中等电子密度,有较多线粒体和溶酶体,扁平囊泡较多。结论:Langerhans细胞在瘢痕疙瘩及增生性瘢痕组织中明显增多,且功能活跃。

食管癌Langerhans细胞和AgNOR的研究

目的 研究食管癌Langerhans细胞和AgNOR与病理临床特征的关系,估价其预后意义。 方法 应用S_(-100)蛋白抗体对56例食管癌组织中Langerhans细胞作免疫组化染色。采用胶质银染色技术对癌细胞AgNOR计量观测。 结果 癌组织中Langerhans细胞密度同淋巴细胞反应密度及癌细胞分化程度呈正相关(P<0.05),癌细胞AgNOR计数与其分化有关,分化愈低,计数愈多。Langerhans细胞和AgNOR均与癌大体类型、浸润深度、淋巴结转移状况及患者性别、年龄等特征无关(P>0.05),与患者预后有关,但二者之间相关无显著性(P>0.05。 结论 反映机体免疫状况的Langerhans细胞和反映癌细胞生物学特性的AgNOR对判断癌细胞分化程度和患者预后有意义,值得进一步研究。