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find Keyword "组织细胞增生症" 8 results
  • Mycobacterium Tuberculosis Infection-associated Hemophagocytic Lymphohistiocytosis: A Case Report and Review of 15 Cases Reported in China

    ObjectiveTo summarize the clinical manifestations,diagnosis and treatment of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. MethodsOne proven diagnosed case of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis was analyzed. Related publications of case reports and articles from Wanfang Data and China Hospital Knowledge Database were also reviewed. ResultsA total of 15 cases were reported,in which 9 cases with complete clinical data. There were 7 males and 2 females,with a mean age of 31.2±15.83 years. The infected tissues were as follows:lung(6 cases),liver(2 cases),spleen(1 case),lymph node(2 cases),pleural(3 cases),peritoneum(2 cases),meninges(1 case),two or more sites infection(5 cases). The most clinical manifestations were fever,hepatosplenomegaly and enlarged lymph glands. The common laboratory abnormalities were liver dysfunction,pancytopaenia and haemophagocytosis in bone marrow. Treatment and outcomes:immunotherapy(5 cases,3 cases with intravenous immunoglobulin and 5 cases with steroid therapy) and anti-tuberculosis (14 cases). 3 cases were cured,6 cases improved and 6 died. ConclusionThere were no specific indexes of mycobacterium tuberculosis infection-associated hemophagocytic lymphohistiocytosis. Early proven diagnosis and administration of anti-tuberculosis and immunotherapy is essentials of effective therapy.

    Release date:2016-08-30 11:31 Export PDF Favorites Scan
  • 朗格汉斯细胞组织细胞增生症漏诊一例

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  • Clinical Analysis on Pulmonary Langerhans’ Cells Histiocytosis and Literatures Review

    ObjectiveTo investigate the clinical features of Pulmonary Langerhans' cells histiocytosis (PLCH). MethodsFour cases of PLCH diagnosed by histopathologic examination between August 2004 and September 2013 were retrospectively analyzed. ResultsFour male patients aged from 19 to 46 year old, including three smokers. The main symptoms were chest tightness, cough, and dyspnea. Pneumothorax was presented in two cases, and tuberculosis was in one. The chest high resolution CT (HRCT) revealed lung cysts, nodles, and reticular changes predominantly in the upper and middle lung fields. The pathological Langerhans' cells infiltration were found in the histological biopsy of lesions of the 4 cases. All of the patients were positive in the immuno-histological staining for the S-100 and CD1a antigens. Two cases were positive in Langrin staining (other two patients didn't underwent the staining). Two of the 4 patients were given oral steroid, and the symptoms were improved in one of them. The case with pulmonary tuberculosis improved in symptoms and CT results showed the absorption of the lesion after anti-tuberculosis therapy. Three cases were not followed up. ConclusionPLCH patients were mainly young adults, often presented with chest tightness, cough, and dyspnea. The clinical features of chest HRCT are bilateral cysts, nodules and reticular changes. The disease may be defined by the finding of pathologic Langerhans' cells or the positive staining for CD1a antigens or Langerin.

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  • 以头痛起病的成人噬血细胞综合征一例

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  • 成人单纯颈椎朗格汉斯组织细胞增生症的临床诊疗

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  • Preliminary study on surgical treatment of Erdheim-Chester disease

    Objective To summarize the preliminary effectiveness of surgical treatment of Erdheim-Chester disease (ECD), so as to improve the understanding of the disease by orthopedic surgeons. Methods The clinical data of 9 patients with ECD between December 2012 and October 2017 were retrospectively analysed. There were 6 males and 3 females with an median age of 42 years (range, 8-61 years). The disease duration was 4-59 months (mean, 39 months). There were 2 cases of multiple lesions, including 1 case involving soft tissue of the buttocks and bilateral tibia, 1 case involving the sinus, skull base, and proximal right tibia; 7 cases with single lesion, including 3 cases of right femoral neck, 1 case of proximal right tibia, 1 case of right humerus, and 2 cases of ribs. Nine patients were diagnosed according to clinical manifestations, imaging examination, and pathological diagnosis. Four patients underwent needle biopsy before operation and 5 patients were diagnosed by postoperative pathology examination. Five cases underwent lesional scraping and internal fixation, 1 case underwent bone scraping and bone grafting, and 3 cases underwent lesion resection. One of the multiple lesions was treated with interferon and hormone. Results Nine patients underwent the surgery safely. There was no fever, wound exudation, infection, etc., and the incisions healed by first intention. All the patients were followed up 4-59 months with an average of 31.4 months. One patient with bilateral tibia and hip soft tissue involvement continued to receive medical treatment, and the tumor was controlled without significant increasing. The remaining 8 patients were examined for X-ray films at 3, 6, and 12 months after surgery, the bone has been fused and the steel plate and intramedullary nail were firmly fixed, and no tumor recurrence was observed. At 1 year after surgery, the pain symptoms of the patients improved and returned to normal life; 3 of them who involving the right femoral neck walked freely, and the quality of life improved significantly. Conclusion ECD patients can achieve the purpose of eliminating lesions and relieving pain after surgical treatment, and the surgical treatment has the advantages of quick relief of pain, improved quality of life, small side effects, and low economic cost when compared with medical treatment.

    Release date:2018-07-30 05:33 Export PDF Favorites Scan
  • 吸烟相关间质性肺疾病的对比分析

    目的提高对不同类型的吸烟相关间质性肺疾病的临床、影像及病理学特点的认识。方法对诊断为呼吸性细支气管炎伴间质性肺疾病(RB-ILD)、脱屑性间质性肺炎(DIP)、肺朗格汉斯组织细胞增生症(PLCH)患者的临床表现、影像学和组织病理学特点进行分析比较。结果3 例患者,男 2 例,女 1 例,年龄 21~71 岁,吸烟史为 4~50 年,均为慢性起病,主要临床表现为咳嗽、咳痰、活动后气促。胸部高分辨 CT 的主要表现为小叶中央型微结节影、网格影、囊状影。病理学表现:RB-ILD 呼吸性细支气管周围少许炎症渗出、管腔中有少许巨噬细胞;DIP 肺泡腔内巨噬细胞聚集;PLCH 以朗格汉斯细胞组成为主的星状结节,朗格汉斯组织细胞表达 CD1α。结论吸烟相关的间质性肺疾病临床及影像表现各异,增强对这类疾病的认识,结合组织病理学检查有利于及早诊断这类疾病。

    Release date:2020-11-24 05:41 Export PDF Favorites Scan
  • 肺朗格汉斯细胞组织细胞增生症误诊为肺肿瘤四例报告并文献复习

    目的探讨误诊为肺部肿瘤的肺朗格汉斯细胞组织细胞增生症(pulmonary Langerhans cell histiocytosis,PLCH)的临床特点,提高该疾病的诊治能力。方法收集本院4例初诊误诊为肺部肿瘤后经病理诊断的PLCH患者的临床资料,并回顾复习相关文献资料进行分析。结果4例PLCH中,男1例,女3例,年龄45~78岁。吸烟2例,不吸烟2例。临床表现2例为体检发现,1例发热,1例浅表淋巴结肿大起病。胸部CT表现为2例多发的肺囊肿、肺大疱改变,4例均出现结节影,其中3例为单发,1例双肺多发圆形小结节影,部分见空洞。3例行正电子发射计算机断层显像(positron emission computed tomography/X-ray computed tomography,PET/CT)检查的患者均提示肺部及淋巴结等病灶标准摄取值增高。4例患者均被误诊为肺部肿瘤,经外科手术、肺穿刺及气管镜等检查,病理见朗格汉斯组织细胞增生,结合S100、CD1a、Langerin等特征性免疫组织化学标志物,最终确诊PLCH。结论 PLCH临床表现不典型,影像学表现为肺结节及多发囊气腔改变需考虑PLCH诊断可能,PET/CT易误诊,通过活检行常规及特征性免疫组织化学病理检查可明确诊断。

    Release date:2023-04-28 02:38 Export PDF Favorites Scan
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