原发性系统性淀粉样变病1例报告并文献复习

目的：探讨原发性系统性淀粉样变病的临床特点和发病机制。方法：结合病例复习文献。结果：发病机制不明确，临床上以心、肾、胃肠道、皮肤等多器官受累为特点，确诊需行组织病理学及免疫组化检查。治疗效果差。结论：早期诊断和早期治疗有可能提高生存率。     

横纹肌溶解症的早期诊断与治疗

目的对横纹肌溶解症的诱因、临床症状，病理特征进行分析，以提高早期诊断率和及时治疗。方法对我们经治的3例、文献报道的相关资料及部分病例进行回顾性分析，总结出常见的致病原因，提出诊断方法、治疗措施。结果横纹肌溶解症是因肌细胞损伤产生毒性物质而导致肾损害的一种疾病。病因多种，血液生化检查以肌酸激酶明显升高为特点。结论了解该病的发病机制，根据病史、临床症状和血液生化检查可早期诊断、及时采取治疗措施。防止并发症发生。     

急性上消化道出血防治的探讨

目的：探讨急性上消化道出血诊断、治疗和预防措施，提高其治愈率，降低死亡率和预防发病。方法：对一组急性上消化道出血病例作回顾性总结分析。结果：全组病例经积极诊断及时治疗．治愈65例，死亡2例，自动出院1例；同时也发现该病有年龄、职业、发病季节和诱因等特点。结论：该病依据病史、症状、体征和辅助检查，一般可以作初步诊断，胃镜检查更具优越性．可做到正确诊断。应重在止血和原发病的治疗，对休克患者应积极进行抗休克治疗．在保守治疗24小时以上仍未控制病情．应及时外科会诊予手术治疗。依据该病发病有年龄、职业、季节和诱因等特点，应加强相关医学知识宣传，可防止其发病，做到预防为主。     

脑淀粉样血管病1例报告并文献复习

目的：探讨脑淀粉样血管病的临床表现及诊断治疗。方法：复习文献结合临床病例分析。结果：该病例的发现逐年增多，且随年龄增高而发病增多。结论：提高临床医师对该病的认识有助于本病的诊断和鉴别诊断及治疗。     

鼻脑型毛霉菌病颅内感染误诊1例并文献复习

目的：阐明鼻脑型毛霉菌病（Rhinocerebral Mucormycosis，RCM）发病的危险因素、误诊原因、临床诊治等要点，以期提高对本病的认识。方法：报告1例头面部外伤后鼻脑型毛霉菌颅内感染死亡病例，并结合文献探讨该病的易感因素、临床特点、诊断治疗方法。结果：对毛霉病的认识不足、原发糖尿病误认为脑外伤所致、病原学检查不及时是延误诊断治疗的主要原因。结论：早期诊断、治疗，使用二性霉素B、及时有效地控制血糖和积极彻底的外科清创手术、高压氧治疗是降低病死率积极有效的方法。     

POEMS综合征(附13例临床分析)

目的：探讨POMES综合征的临床特点，提高诊治水平。方法：对13例POEMS综合征临床资料进行回顾分析。结果：本组临床特征与国外组基本一致。不同点为本组病例以周围神经病为突出表现，常伴植物神经损害；视乳头水肿、脾肿大多见。性腺机能减退较明显，甲状腺功能低下多见而临床症状不明显；淋巴结肿大少见；M蛋白检出率较低。结论：本组临床特征与国外组基本相似，但有其特点，早期极易误诊，免疫抑制剂治疗有效。病因和发病机制尚不清楚。     

多发性骨髓瘤细胞遗传学异常的研究进展

分子细胞遗传学技术的进步推动了多发性骨髓瘤的细胞遗传学研究，发现了该病遗传学改变的一些特点和规律，并发现某些遗传学改变与疾病发病机制、疾病临床表现等有密切关系。这些发现不仅有利于该病发病机制的研究，也为临床预后评估、指导治疗提供参考，同时为开发新疗法提供线索。     

肺隐球菌病(附2例报告)

目的：通过总结肺隐球菌病的临床特点以引起医务工作者对此病的重视。方法：结合本院2例肺隐球菌病，总结该病的临床特点，诊断方法和治疗方案，结果：肺隐球菌病的发病率有所上升，症状无特征性，确诊主要靠病理检查，该病是否需要治疗意见尚未统一，结果：由于该病的临床表现不典型及影象学表现的多样性，易导致误诊，对治疗方案尚需通过较多病例的观察后得出结论。     

肺出血-肾炎综合征1例报告并文献复习

目的 :探讨肺出血 -肾炎综合征的临床特点。方法 :结合病例复习文献。结果 :该病的发生与流感病毒、接触汽油、松节油甚至吸烟、混凝土粉尘有关。发病机理与肺泡基底膜、肾小球基底膜共同形成抗原抗体复合物有关。发病以青年男性多见 ,临床特征为咯血、贫血、尿检异常及肾功进行性减退。胸片异常者只有 2 0 %左右。治疗包括血浆置换、激素、免疫抑制剂的应用等。结论 :对该病应加强认识 ,早期诊断、早期治疗 ,才能提高生存率。     

血管内大B细胞淋巴瘤1例报告及文献复习

目的：了解血管内大B细胞淋巴瘤的临床，病理特征及治疗、预后情况。方法：对1例血管内大B细胞淋巴瘤（IVL）的临床特点及病理特征结合国内外文献进行分析。结果：该病例通过对肌肉活检得到诊断并给予积极的治疗。结论：IVL预后一般较差，但通过提高该病的诊断水平，可于肌肉活检做出诊断，做到早期诊断，及时治疗，可改善预后争取较好的转归。     

重症副肿瘤性天疱疮伴多种细菌感染患者PICC置管的护理

报告了对1例重症副肿瘤性天疱疮伴多种细菌感染患者进行PICC的护理,该患者未发生继发感染,病情得到了有效控制,痊愈出院.认为密切观察病情、针对患者皮肤感染情况采取个性化护理、及时观察疗效等措施对患者的病情恢复十分重要.     

Castleman病引起副肿瘤性天疱疮的系统性损害

目的:分析伴发Castleman病的副肿瘤性天疱疮系统性损害和临床治疗效果。方法:回顾性分析3例伴Castleman病的副肿瘤性天疱疮患者的临床资料。结果:除皮肤、黏膜损害,患者还有呼吸、消化、神经等多系统受累。手术切除Castleman瘤后皮损好转,但术后呼吸和消化系统症状均加重。结论:伴发Castleman病的副肿瘤性天疱疮可出现多系统损害,手术切除Castleman瘤后皮损改善,但其他系统损害短期内可加重。     

Oral lichenoid tissue reactions: diagnosis and classification

The concept of lichenoid tissue reaction/interface dermatitis was introduced in dermatology to define a number of diverse inflammatory skin diseases linked together by the presence of common histopathological features. Similarly to the skin, the oral mucosa is affected by a variety of oral lichenoid lesions. Oral LTRs (OLTRs) include: oral lichen planus; oral lichenoid contact lesion; oral lichenoid drug reaction; oral lichenoid lesions of graft-versus-host disease; oral discoid lupus erythematosus; oral lesions of systemic lupus erythematosus; erythema multiforme; paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome; chronic ulcerative stomatitis and lichen planus pemphigoid. Traditionally, diagnosis of OLTRs relies on clinical and histological correlation but in several instances this approach fails to provide a reliable diagnosis. Inclusion of molecular techniques may refine our ability to differentiate OLTRs.     

Human autoantibodies against desmoplakins in paraneoplastic pemphigus.

Recently, a previously unrecognized autoantibody mediated blistering disease, paraneoplastic pemphigus has been described. Paraneoplastic pemphigus is associated with lymphoid malignancies, thymomas, and poorly differentiated sarcomas. Serum of affected patients contain pathogenic autoantibodies that immunoprecipitate from normal keratinocytes a characteristic complex of four polypeptides with M(r) of 250, 230, 210, and 190 kD. As our preliminary studies indicated that the 250-kD and the 210-kD antigens comigrated with desmoplakins I and II, we investigated the possibility that autoantibodies against the desmoplakins were a component of this autoimmune syndrome. 11 sera from affected patients were tested by indirect immunofluorescence against desmosome containing tissues, immunoprecipitation of metabolically labeled keratinocytes, and Western immunoblotting of desmoplakins I and II that had been purified to homogeneity from pig tongue epithelium. By indirect immunofluorescence, 9 of 11 sera showed strong binding to epithelial and nonepithelial desmosomes, and 2 were weakly reactive. All 11 immunoprecipitated 250- and 210-kD bands of variable intensity that comigrated with bands identified by a murine monoclonal antidesmoplakin antibody, and immunoblotting confirmed binding of the serum autoantibodies to purified desmoplakins. This demonstrates that paraneoplastic pemphigus is the first human autoimmune syndrome in which autoantibodies against the desmoplakins are a prominent component of the humoral autoimmune response.     

Autoimmune bullous dermatoses: a review

Bullous dermatoses can be debilitating and possibly fatal. A selection of autoimmune blistering diseases, including pemphigus vulgaris, paraneoplastic pemphigus, bullous pemphigoid, cicatricial pemphigoid, dermatitis herpetiformis and linear IgA dermatosis are reviewed. Pemphigus vulgaris usually starts in the oral mucosa followed by blistering of the skin, which is often painful. Paraneoplastic pemphigus is associated with neoplasms, most commonly of lymphoid tissue, but also Waldenstr?m's macroglobulinemia, sarcomas, thymomas and Castleman's disease. Bullous pemphigoid is characterized by large, tense bullae, but may begin as an urticarial eruption. Cicatricial (scarring) pemphigoid presents with severe, erosive lesions of the mucous membranes with skin involvement in one third of patients focused around the head and upper trunk. Dermatitis herpetiformis is intensely pruritic and chronic, characterized by papulovesicles and urticarial wheals on the extensor surfaces in a grouped or herpetiform, symmetric distribution. Linear IgA dermatosis is clinically similar to dermatitis herpetiformis, but it is not associated with gluten-sensitive enteropathy as is dermatitis herpetiformis.     

1例副肿瘤型天疱疮合并Castleman病患儿的护理

总结1例副肿瘤型天疱疮合并Castleman病患儿的护理体会。护理要点包括：皮肤黏膜完整性受损的护理、密切观察药物的作用及不良反应、心理护理等。经过精心的治疗和护理,患儿全身散在皮疹基本结痂,口腔内破损较前好转,双手皮疹结痂脱落,好转出院。     

副肿瘤性天疱疮的超声及CT特征

目的 探讨副肿瘤性天疱疮（PNP）的超声及CT特征与临床表现。方法 回顾性分析手术病理证实的8例副肿瘤性天疱疮患者完整的临床、实验室、影像学、病理学及术后随访资料。结果 8例副肿瘤性天疱疮主要临床特征和超声、CT表现综合归纳为：多形性皮肤损害伴疼痛性黏膜糜烂溃疡，同时合并内脏软组织肿块。肿瘤部位：腹膜后6例，胸腔纵隔2例。病理类型：巨淋巴结增生7例，非霍奇金淋巴瘤1例。结论 超声及CT可以发现副肿瘤性天疱疮时皮肤黏膜损害出现之前的内脏肿瘤，且应首选腹部超声检查，其次是胸部CT。     

Paraneoplastic syndrome in kidney cancer

In a prospective study 60 patients suffering from histologically confirmed renal cell carcinoma were examined on paraneoplastic alterations. The following parameters were determined: Stauffer-syndrome (BSG, total protein with serum electrophoresis, alcaline phosphatase, gamma-GT, Quick values), total and ionized calcium, parathormone, calcitonin, hemoglobin, thrombocytes, LDH, IgE and arterial blood pressure. Compared to the findings obtained by other authors, we saw paraneoplastic syndromes only rarely. There was no correlation found to the stage of the tumor or to prognosis. In only four out of 60 patients paraneoplastic led to the diagnosis "renal carcinoma". With the exception of BGS, which was raised in 75% of the patients, paraneoplastic parameters are not suitable for the screening or the early diagnosis of renal carcinomas.     

Paraneoplastic Syndromes: An Approach to Diagnosis and Treatment

Recent medical advances have improved the understanding, diagnosis, and treatment of paraneoplastic syndromes. These disorders arise from tumor secretion of hormones, peptides, or cytokines or from immune cross-reactivity between malignant and normal tissues. Paraneoplastic syndromes may affect diverse organ systems, most notably the endocrine, neurologic, dermatologic, rheumatologic, and hematologic systems. The most commonly associated malignancies include small cell lung cancer, breast cancer, gynecologic tumors, and hematologic malignancies. In some instances, the timely diagnosis of these conditions may lead to detection of an otherwise clinically occult tumor at an early and highly treatable stage. Because paraneoplastic syndromes often cause considerable morbidity, effective treatment can improve patient quality of life, enhance the delivery of cancer therapy, and prolong survival. Treatments include addressing the underlying malignancy, immunosuppression (for neurologic, dermatologic, and rheumatologic paraneoplastic syndromes), and correction of electrolyte and hormonal derangements (for endocrine paraneoplastic syndromes). This review focuses on the diagnosis and treatment of paraneoplastic syndromes, with emphasis on those most frequently encountered clinically. Initial literature searches for this review were conducted using PubMed and the keyword paraneoplastic in conjunction with keywords such as malignancy, SIADH, and limbic encephalitis, depending on the particular topic. Date limitations typically were not used, but preference was given to recent articles when possible.ADH = antidiuretic hormone; CSF = cerebrospinal fluid; CT = computed tomography; IL = interleukin; IV = intravenous; IVIG = IV immunoglobulin; LEMS = Lambert-Eaton myasthenia syndrome; NICTH = non–islet cell tumor hypoglycemia; PNS = paraneoplastic neurologic syndrome; PTH = parathyroid hormone; PTHrP = PTH-related protein; SIADH = syndrome of inappropriate antidiuretic hormone secretionMore than 100 years ago, it was recognized that certain cancers cause various symptoms not attributable to direct tumor invasion or compression.¹ Labeled paraneoplastic syndromes in the 1940s,² these conditions remained poorly understood until recently. Currently, the best described paraneoplastic syndromes are attributed to tumor secretion of functional peptides and hormones (as in the case of endocrine paraneoplastic syndromes) or immune cross-reactivity between tumor and normal host tissues (as in the case of neurologic paraneoplastic syndromes). During the past several years, medical advances have not only improved the understanding of paraneoplastic syndrome pathogenesis but have also enhanced the diagnosis and treatment of these disorders.Effective diagnosis and treatment of paraneoplastic syndromes may substantially affect overall clinical outcomes. In some instances, paraneoplastic syndromes are manifest before a cancer diagnosis. Thus, their timely recognition may lead to detection of an otherwise clinically occult tumor at an early and highly treatable stage. Such a scenario occurs most commonly with neurologic paraneoplastic disorders. Although considerable clinical overlap with nonparaneoplastic disorders has long confounded the diagnosis of these conditions, numerous serologic and radiographic studies are currently available to aid in this process.It is estimated that paraneoplastic syndromes affect up to 8% of patients with cancer.³ As patients with cancer live longer, and as diagnostic methods improve, this prevalence will likely increase. Yet, given the rarity of individual paraneoplastic syndromes, there are few prospective clinical trials to guide management. However, paraneoplastic syndromes frequently represent subtypes of conditions that also occur outside of a cancer association. This review incorporates clinical experience from case series of specific paraneoplastic disorders, as well as larger studies of clinically similar, nonparaneoplastic conditions, to provide an overview of the diagnosis and treatment of the most commonly encountered paraneoplastic syndromes.     

抗Hu副肿瘤综合征病例2例分析并文献复习

目的:探讨抗Hu神经系统副肿瘤综合征的临床特点及预后。方法:报道我院2例抗Hu副肿瘤综合征并结合文献进行回顾性分析。结果:其中1例表现为严重的中枢性低通气,同时伴有周围神经病变,但全身PET-CT并未发现肿瘤;另1例表现为非特异性症状,肿瘤筛查中发现头颈部肿块,主要分布在鼻窦和腮腺,病理活检为小细胞癌。鼻窦肿块切除后短期内患者症状缓解明显。搜索既往报道的抗Hu相关副肿瘤综合征患者477例,最常见的表现是亚急性感觉性周围神经病,颅脑的MRI表现无特异性,最常见合并小细胞肺癌;在所有的病例中,有2.5%的患者表现为中枢性低通气。结论:抗Hu副肿瘤综合征应作为中枢性低通气的鉴别诊断之一。副肿瘤综合征相关抗体的检测,有利于更早发现肿瘤。