Eosinophilic granuloma of the sternum in a child treated with closed biopsy

Langerhans cell histiocytosis is a rare neoplastic proliferative disorder of the Langerhans cells. The clinical course is variable, ranging from a low symptomatic single bone lesion to fatal multiple organ involvement. Rarely, the sternum can be the first and single location of the disease. We report on a 12‐year‐old boy who presented with an aggressive lytic lesion of the proximal sternum associated with local pain and afternoon fever. Histopathological analysis of the closed biopsy specimen indicated eosinophilic granuloma of bone/Langerhans cell histiocytosis. Soon after the biopsy procedure the pain and fever subsided. Computed tomography at 2 months showed healing of the lytic lesion. The patient received no other type of treatment. At 2 year follow up he was symptom and disease free.     

Ultrasonographic features of Langerhans cell histiocytosis of the thyroid

Langerhans cell histiocytosis (LCH) is a rare disease, especially when it involves the thyroid gland. Awareness of ultrasonic features will be helpful for a clinician who should consider this disease in the differential diagnosis from other more common thyroid disorders, especially prior to surgery. Here, we report two patients who have histologically confirmed LCH of the thyroid and summarize the reported cases with ultrasonographic scans from the last 10 years (n=10). Ultrasonograms showed isolated or multiple hypoechoic nodules in unilateral or bilateral thyroid gland. Internal acoustic features of most nodules was heterogeneous (n=5) or hypoechoic (n=2).     

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。     

36例口腔颌面-头颈部朗格汉斯细胞组织细胞增多症临床及预后分析

目的:探讨口腔颌面-头颈部朗格汉斯细胞组织细胞增多症(Langerhans cell histiocytosis,LCH)的临床特征、诊断、治疗方法及预后,为临床治疗提供更强的循证医学证据.方法:回顾分析上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科收治的36例成人及儿童口腔颌面-头颈部朗格汉斯细胞组织细胞增多...     

免疫抑制剂对人胰岛细胞的毒性作用

目的 探讨临床常用免疫抑制剂对人胰岛细胞的直接毒性作用.方法 应用体外分离纯化后培养的人胰岛细胞,以刺激系数和丫啶橙/碘丙啶染色为效应指标,检验低、中、高不同浓度免疫抑制剂对人胰岛细胞的直接毒性作用.结果 体外培养3天后,低浓度免疫抑制剂未表现出胰岛毒性作用;中浓度免疫抑制剂对胰岛的毒性不同,以他克莫司为重;高浓度免疫抑制剂有较强的胰岛毒性.结论 免疫抑制剂对人胰岛细胞直接毒性作用与浓度相关,选择低胰岛毒性免疫抑制方案有助于提高胰岛移植成功率.     

慢性特发性组织细胞增多症一例

患者男,34岁。因双侧腋部皮肤溃疡久不愈合,伴疼痛1年,胸闷、乏力、多尿3年就诊。具有尿崩症、突眼、皮肤黏膜病变（包括浸润结节和溃疡斑块,特别是在腋下,生殖器区域）。其他尚见广泛的皮疹、脱屑或结痂性丘疹。组织学特点为真皮全层弥漫分布的朗格汉斯组织细胞。瘤细胞表达CDla、S-100。组织病理及免疫组化检查符合朗格汉斯组织细胞增生症（Hand—Schuller-Christian病）。本病目前尚无特异性的治疗方法。多系统病变采取化疗,单发病灶者手术及放疗疗效好。     

Multisystem Langerhans cell histiocytosis: Literature review and case report

Langerhans cell histiocytosis (LCH) refers to a group of diseases of unknown etiology, typically discovered in childhood, characterized by the accumulation of Langerhans cells (white blood cells with large cell nuclei that may contain cytoplasmic histiocytosis X bodies) involving one or more organ systems, including bones, lungs, pituitary gland, skin, lymph nodes, and liver. This disease is also known as histiocytosis X or eosinophilic granuloma. Pulmonary LCH is common (identified in 40% of LCH patients) and may be isolated to the lung or involve other organs. Although LCH is characterized by clonal cell proliferation, adult LCH is considered likely to represent the manifestation of an aberrant immune response to an unspecified antigenic stimulus rather than a manifestation of tumor proliferation. We report a very complicated clinical case of LCH, with multiple organ damage that received a variety of different diagnoses. An LCH diagnosis was confirmed based on postoperative spinal cord pathology results and immunohistochemistry examinations. This case report highlights the clinical, laboratory, and imaging signs observed in this case that should be noted to help doctors more quickly recognize, diagnose, and treat similar cases.     

眼眶朗格汉斯细胞组织细胞增生症的MRI表现特点分析

目的：探讨眼眶朗格汉斯细胞组织细胞增生症(LCH)的 MRI 表现特征,提高其诊断准确性。方法回顾性分析经术后病理证实的14例眼眶 LCH 患者的临床及 MRI 资料。结果9例患者以眼睑肿胀或伴炎症表现、眼突、眼部肿物就诊,本组中1例伴有明显咳嗽、咳痰,1例伴有多饮多尿症状。右眶病变7例,左眶病变6例,双眶同时受累1例。外上壁交界处病灶6例,外侧壁病变5例,上壁病变3例。MRI 表现：13例 T1 WI 上呈等、低信号,11例 T2 WI 上呈不均匀等、高信号,多数信号不均匀。嗜酸性肉芽肿病灶边界较清楚,其他类型多边界不清。增强后病变多数呈较明显不均匀强化。结论MRI 可清楚、准确地显示 LCH 病变范围,结合临床表现有助于本病早期诊断,MRI 可为临床制订手术方案、治疗计划提供可靠依据。