中枢神经细胞瘤11例临床病理及免疫组化特点

目的 提高对中枢神经细胞瘤（ＣＮＣ）的临床病理及免疫组化特征的认识,减少漏诊和误诊。方法 对１１例ＣＮＣ进行临床病理分析及免疫组化染色。结果 １１例ＣＮＣ的组织学特点：肿瘤由密集均匀一致的少突胶质细胞瘤样小圆细胞和成片的无核纤细原纤维岛间质组成。未见异形增生及坏死。免疫组化１１例Ｓｙｎ均阳性（１００％）;ＮＳＥ３／１１例阳性（２７．３％）;Ｌｅｕ７／１１例阳性（９．１％）;ＧＦＡＰ和Ｓ１００蛋白肿     

胃黄斑瘤10例临床病理分析

胃黄斑瘤 (ｘａｎｔｈｅｌａｓｍａ)又称黄色瘤 (ｘａｎｔｈｏｍａ)或脂质岛(ｌｉｐｉｄｉｓｌａｎｄｓ) ,是一种在胃黏膜内形成的黄白色瘤样斑块 ,属于良性病变 ,胃镜检查常因缺乏对本病的认识而漏诊 ,病理组织学诊断时需与胃印戒细胞癌相鉴别。现将作者在胃黏膜活检中诊断的 10例黄斑瘤结合文献就其临床病理特点报道如下。1　材料与方法收集我院病理科 1998 3— 2 0 0 1 8间 ,胃镜活检组织诊断的10例黄斑瘤。标本常规固定、包埋、切片 ,每例均做ＨＥ染色和ＰＡＳ/ＡＢ组化染色 ,其中 7例行免疫组化染色 (ＳＰ法 )。抗体ＣＤ6 8、Ｓ 10 0、…     

颅内原发性未成熟畸胎瘤病理学观察

颅内原发性未成熟畸胎瘤 (ｉｎｔｒａｃｒａｎｉａｌｐｒｉｍａｒｙｉｍｍａｔｕｒｅｔｅｒａｔｏｍａ ,ＩＰＩＴ)是相当少见的肿瘤 ,现将作者近 30年尸检中遇到的 7例 ,结合临床病理组织形态及免疫组化表达情况进行的观察 ,报道如下。1　材料与方法收集我院病理科 196 8～ 1998年尸检 76 3例中 7例ＩＰＩＴ存档蜡块 ,组织常规制片 ,ＨＥ染色 ,光镜观察。免疫组化染色采用枸橼酸—微波—ＳＰ法。选用抗体有甲胎蛋白 (ＡＦＰ)、癌胚抗原 (ＣＥＡ)、胎盘碱性磷酸酶 (ＰＬＡＰ)、绒毛膜促性腺激素 - β(ＨＣＧ - β)、波形蛋白 (ｖｉｍｅｎｔｉ…     

四川地区间变性大细胞淋巴瘤的临床病理与免疫组化研究

目的 观察四川地区间变性大细胞淋巴瘤（ａｎａｐｌａｓｔｉｃ ｌａｒｇｅ ｃｅｌｌ ｌｙｍｈｏｍａ,ＡＬＣＬ）的临床病理和免疫组化特征。方法 搜集临床资料并随访,应用免疫组化（ＡＢＣ法）,对１９例ＡＬＣＬ的临床病理和免疫组化进行研究。结果 记均阳性）。１１例为ＣＤ４５阳性,１０例ＥＭＡ阳性,１４例ＴＩＡ１阳性。ＣＤ１５及ＣＤ６８均为阴性。形态学上瘤组织表现为窦、滤泡间侵犯,瘤细胞形态多样,可见胚胎样     

5例原发性肺平滑肌肉瘤的临床病理分析

肺部原发性平滑肌肉瘤少见 ,现报告 5例如下。1　材料与方法病例选自 1984～ 1998年间手术切除标本 ,3例来自本院 ,2例来自淮北市人民医院。标本经 10 %福尔马林固定 ,常规石蜡切片 ,ＨＥ染色 ,光镜观察 ,并做免疫组织化学染色(Ｄｅｓｍｉｎ ,Ａｃｔｉｎ ,ｖｉｍｅｎｔｉｎ ,ＣＤ6 8,ＥＭＡ及Ｓ 10 0 )。免疫组化染色用ＡＢＣ法 ,所用试剂均购自北京中山生物技术有限公司。2　结果2 .1　临床资料　男 4例 ,女 1例 ,平均年龄 49 6岁。其临床病理资料见表 1。表 1　平滑肌肉瘤临床病理资料ｎ 年龄(岁 ) 性别术前病程临床症状肿瘤部位 肿瘤大小…     

c-myc基因产物及蛋白激酶C在膀胱癌组织中的表达意义及相关性研究

笔者应用ＳＡＢＣ免疫组化染色方法研究了ｃ-ｍｙｃ及ＰＫＣ在膀胱癌中的表达情况,并分析了二者的表达与膀胱癌多种临床,病理特点之间的关系。１村料与方法１９９３年～１９９５年间手术切除的膀胱移形细胞癌石蜡包埋标本４８例,其中男３７例,女１１例,年龄为２９岁～７５岁之间,平均５７．３岁。小鼠抗人ｃ-ｃｍｙ多克隆抗体(１:５０)和小鼠抗人ＰＫＣ抗体(１:１００),ＳＡＢＣ试剂盒(ＢＯＳＴＥＲ公司)。链酶亲合素-生物素化过氧化物酶(ＳＡＢＣ)法免疫组化染色。ＰＢＳ代替一抗作空白对照。６例膀胱正常粘膜作正常对照。ＤＡＢ显色。每张…     

淋巴瘤样丘疹病

目的 研究淋巴瘤样丘疹（ＬｙＰ）的临床与病理特征,以提高ＬｙＰ的临床早期诊断率。方法 运用组织病理学及免疫组化标记（ＳＰ或ＡＢＣ）法对７例ＬｙＰ进行临床、病理学和免疫组化（ＣＤ２０、ＣＤ４５ＲＯ、ＣＤ３０、ＣＤ６８及ＨＴＬＶ－１抗体）观察。结果 临床表现为患者的躯干和／或四肢皮肤多发性、复发性、小于１ｃｍ的斑丘疹。临床上是一个慢性、良性经过,而病理组织学ＬｙＰ是由多量小或大的异型淋巴样细胞组成,淋     

脑原发性恶性淋巴瘤免疫组化及病理形态学分析

总结６例脑原发性恶性淋巴瘤，分析其临床症状及光镜，电镜，免疫组化特点，结果表明颅内多发病灶及颅压增高为临床特征，组织学特点为弥漫分布，围绕血管排列，ＬＣＡ，Ｌ２６，ＵＣＨＬ１，ＧＦＡＰ，ＥＭＡ，ＮＳＥ等免疫组织化学染色及１例电镜检查证实为Ｂ细胞性，并对鉴别诊断作了讨论。     

P—170在亲本S—180和抗药S—180—R瘤细胞中的表达

目的探讨ＭＤＲ基因产物Ｐ－糖蛋白（Ｐ－１７０）在亲本Ｓ－１８０及抗阿霉素Ｓ－１８０－Ｒ瘤细胞中的表达，（２）方法，用ＡＢＣ免疫组化方法，按日本ＤＡＫＯ公司免疫药盒程序进行，单抗为Ｃ２１９，ＬｉｎＫｉｎｇｒｅａｇｅｎｔ１：４００，Ｓｔｒｅｐｔａｉｖｄｉｎｒｅｇａｇｅｎｔ１：４００，ＤＡＢ染色，苏木精复染。（３）结果，Ｓ－１８０－Ｒ瘤细胞中，有明显的Ｐ－１７０表达，亲本Ｓ－１８０细胞则无此表达，抗阿抗     

18例脉络丛肿瘤的临床病理观察

脉络丛肿瘤属颅内较少见的一种肿瘤。本文对 18例脉络丛肿瘤的临床病理特点进行了回顾总结 ,并对其超微结构和免疫组化特征作初步探讨。1　材料与方法复习我院 1986～ 1997年间 18例病理诊断为脉络丛乳头状瘤 (Ｃｈｏｒｏｉｄｐｌｅｘｕｓｐａｐｉｌｌｏｍａｓ,ＣＰＰ)和脉络丛癌 (Ｃｈｏｒｏｉｄｐｌｅｘｕｓｃａｒｃｉｎｏｍａｓ,ＣＰＣ)的临床病理资料 ,同时用ＳＰ法对所有病例的石蜡包埋切片进行免疫组化染色。使用的抗体包括 :角蛋白 (ＣＫ)、上皮膜抗原 (ＥＭＡ)、癌胚抗原 (ＣＥＡ)、胶质纤维酸性蛋白 (ＧＦＡＰ)、波形蛋白 (Ｖｉｍ)…     

Acid beta-glucosidase and the diagnosis of Gaucher's disease in liver and spleen.

Variable amounts of residual 4-methylumbelliferyl-beta-glucosidase activity gave rise to difficulties in the diagnosis of Gaucher's disease using spleen and particularly liver. Soluble and particulate components of beta-glucosidase, which interfere with the diagnosis, may be eliminated by preincubation of homogenates at pH 3.0 or with 100 mM sodium chloride at pH 4.0. After either of these treatments the optimum of acid beta-glucosidase was pH 4.5 and diagnosis of Gaucher's disease could be more easily made using homogenates of spleen and liver. There was residual acid beta-glucosidase activity in one of the Gaucher livers.     

Whipple's disease without malabsorption: new atypical features

The diagnosis of Whipple's disease in the absence of intestinal involvement is difficult and often overlooked. We describe five patients aged 8-71 years with normal jejunal biopsies and disparate clinical features, previously unrecognized in Whipple's; all were investigated at a single institution over a period of 18 months. Routine histological examination for periodic acid-Schiff (PAS) positive macrophages and polymerase chain reaction (PCR) analysis for Tropheryma whippelii was performed on the small intestine in all patients. PCR analysis was also performed on various tissues including peripheral blood, lymph node, muscle, synovium and spleen in individual patients. Patients 1, 2, 4 and 5 had unusual presenting features not previously associated with Whipple's: intractable immune thrombocytopenic purpura (ITP), juvenile chronic arthritis, isolated muscle weakness and quadriparesis, respectively. Patient 3 presented with pyrexia of unknown origin. All patients had histologically normal small-bowel biopsies with no evidence of PAS positive macrophages. PCR for T. whippelii was positive in all patients in one or more tissues: peripheral blood, intestine, muscle, lymph node and synovium. PAS- positive macrophages were found in 4/5 patients in various sites: lymph node, muscle, spinal cord. Whipple's disease presents with protean clinical features and should be considered in granulomatous disorders of unknown aetiology even in the absence of gastrointestinal involvement.      

Elevated serum and spleen angiotensin converting enzyme and serum lysozyme in Gaucher's disease.

In adult chronic non-neuronopathic (Type 1) Gaucher's disease significant (p less than 0.001) elevations of angiotensin converting enzyme in serum (93.3 +/- 14.8 nmol/min/ml; number elevated, 8/11; normal control 32.2 +/- 1.30, n = 58) and spleen (5.62 +/- 0.35 nmol/min/mg protein, n = 2; control, 0.431 +/- 0.101, n = 4) and serum lysozyme (15.6 +/- 3.37 mug/ml; number elevated, 4/5) were observed. The KM for hippuryl-L-histidyl-L-leucine of Gaucher (1.31 mM) and normal (1.23 mM) serum angiotensin converting enzyme were similar. The increased angiotensin converting enzyme (ACE) in Gaucher's disease may be related to the genetic defect resulting in increased ACE synthesis in Gaucher cells, or perhaps generally, while high lysozyme may reflect an increased body mass of reticuloendothelial cells. These enzyme elevations may be of use in suggesting the possible presence of Gaucher's disease and perhaps in assessing the magnitude of pathologic involvement.     

Comparison of the tartrate-resistant acid phosphatase in Gaucher's disease and leukemic reticuloendotheliosis

Tartrate-resistant acid phosphatase was isolated from serum and spleen of patients affected by Gaucher's disease. Electrophoretic and antigenic properties were compared to the enzyme isolated from hairy cells described in a previous study (9). The enzyme isolated from Gaucher serum has electrophoretic and antigenic properties identical to the acid phosphatase band 5b of hairy cells. The major tartrate-resistant acid phosphatase in the Gaucher spleen is band 5a. Bands 5a and 5b have identical protein structure indicated by their identical antigenicity. The removal of carbohydrate from band 5a by sialidase converted band 5a to 5b.     

腺泡状软组织肉瘤28例临床病理分析

目的探讨腺泡状软组织肉瘤(ASIS)的临床病理特征及鉴别诊断。方法对28例ASPS进行HE染色，光镜观察，其中18例做组化(PAS)和免疫组化(SP法)染色。结果28例腺泡状软组织肉瘤中，男性19例，女性9例；年龄2～55岁，平均29．5岁；病变主要位于下肢深部骨骼肌组织内。组织学可见瘤组织呈特征性的腺泡或巢团样排列，胞质内可见嗜伊红颗粒及结晶体，PAS染色( )。免疫组化：18例中，5例S-100蛋白弥漫( )，3例NSE局灶( )，3例Vim局灶( )，desmin、SMA、EMA、Myo、aetin和CK均(一)。结论腺泡状软组织肉瘤多见于年轻男性，结合临床和病理特征，可作出正确的病理诊断。     

Fibromyalgia and Gaucher's disease

BACKGROUND: Patients with symptomatic Gaucher's disease sometimes have non-specific symptoms (such as general malaise with widespread musculoskeletal pains) that respond poorly to enzyme replacement treatment. These may indicate fibromyalgia syndrome; if so, other therapeutic options might be more appropriate. AIM: To identify patients with Gaucher's disease for whom fibromyalgia-specific therapy may be therapeutic. DESIGN: Questionnaire-based survey. METHODS: Adult patients (n = 109) with non-neuronopathic Gaucher's disease and adult healthy controls (n = 108) completed health-related questionnaires including the Fibromyalgia Impact Questionnaire, and underwent testing with a dolorimeter to ascertain sensitivity at 22 tender points. RESULTS: Six patients, but no controls, met the criteria for fibromyalgia. Patients with fibromyalgia had a significantly greater incidence of co-morbidities (p = 0.014) relative to other patients with Gaucher's disease; four suffered from bone involvement and were receiving enzyme therapy, but two were untreated. DISCUSSION: The presence of fibromyalgia-specific trigger points may result from multiple aetiologies, or may be an independently-sorting predisposition. Our findings cannot distinguish between these possibilities, but if fibromyalgia were the cause, enzyme replacement therapy would be expensive and inappropriate.     

Acid hydrolases in serum from patients with lysosomal disorders

The activity of acid hydrolases was studied in serum from patients with mucolipidosis (II and III) and other lysosomal disorders. In mucolipidosis II and III all hydrolases examined except alpha-glucosidase, beta-glucosidase and acid phosphatase were greatly increased. High values for beta-galactosidase were seen in mucopolysaccharidosis types I and II, Gaucher's disease, juvenile amaurotic idiocy and metachromatic leucodystrophy. N-Acetyl-beta-glucosaminidase activity was high in mucopolysaccharidosis types I, II, III and Gaucher's disease. The activity of beta-glucuronidase was increased in mucopolysaccharidosis types I, II and III, Gaucher's disease, juvenile amaurotic idiocy and metachromatic leucodystrophy. Acid phosphatase had increased activity only in Gaucher's disease. In several lysosomal storage disorders no increased values could be found. It is suggested that high values in serum from patients with lysosomal storage disorders (not including mucolipidosis II and III) may depend upon liver cell damage, which disturbs the clearing of acid hydrolases from serum.     

Pro-inflammatory cytokines and the pathogenesis of Gaucher's disease: increased release of interleukin-6 and interleukin-10

Gaucher's disease is characterized by hepatosplenomegaly, bone-marrow infiltration, osteonecrosis and bone thinning, associated with the presence of pathological macrophages that contain undegraded glycosphingolipids. To investigate the possible role of cytokines in the systemic and local manifestations of established Gaucher's disease, interleukin-1 beta (IL-1 beta), interleukin-6 (IL-6), tumour necrosis factor-alpha (TNF alpha) and interleukin-10 (IL-10) were measured in freshly-separated serum. Samples from eight male and 14 female patients with type 1 Gaucher's disease were compared with sera from 22 healthy age- and sex-matched controls. Concentrations of IL-6 and IL-10 were significantly elevated in sera from patients with Gaucher's disease (11.9 +/- 1.8 (SEM) pg/ml and 5.4 +/- 0.5 (SEM) pg/ml, respectively) compared with those of controls (4.1 +/- 0.9 (SEM) and 0.8 +/- 0.3 (SEM) pg/ml, p < 0.0001). No significant differences in concentrations of TNF alpha or IL-1 beta were identified. IL-6 has been implicated in the development of localized osteolysis in multiple myeloma and in the development of post-menopausal osteoporosis. High concentrations of IL- 6 in the serum of patients with Gaucher's disease may thus reflect the development of the bone lesions commonly associated with this disorder. Since IL-6 and IL-10 are important regulators of lymphocyte growth and differentiation, and IL-6 concentrations were significantly raised in patients with oligo- or polyclonal increases in serum immunoglobulins, enhanced release of these cytokines from pathological macrophages provides a pathological link between Gaucher's disease and associated lympho-proliferative disorders.      

Antibodies to porcine uteroferrin used in measurement of human tartrate-resistant acid phosphatase

The immunological similarity between human tartrate-resistant acid phosphatase (EC 3.1.3.2) and porcine uteroferrin previously reported for the isoenzyme from spleens of patients with leukemic reticuloendotheliosis (Ketcham et al., J Biol Chem 1985;260:5768-76) has been confirmed for partly purified acid phosphatase found in the spleen of a patient with Gaucher's disease, and for the corresponding isoenzyme in other tissues and serum. Anti-uteroferrin antibodies raised in rabbits have been used to demonstrate the feasibility of their application in an immunoassay for tartrate-resistant acid phosphatase in serum.     

Gaucher's disease

Two cases of Gaucher's disease are described. The diagnosis was based on clinical appearance, observation of the typical cells in the sternal and splenic puncture biopsies. Leucocytic beta-glucocerebrosidase activity was found inhibited in contrast to normal activity of the other lysosomal glycosidases. A female of 51 developed significant changes in the bones, progressive enlargement of the liver, chronic calculous cholecystitis 36 years after splenectomy. Current aspects of pathogenesis, clinical symptoms, diagnosis and treatment of Gaucher's disease are reviewed.