T细胞淋巴瘤1和CD44蛋白在Burkitt淋巴瘤中的表达及其诊断价值

目的 探讨T细胞淋巴瘤1(TCL1)和CD44蛋白在Burkitt淋巴瘤中的表达及其诊断价值.方法 在石蜡包埋的实验组25例Burkitt淋巴瘤和对照组25例非特指弥漫性大B细胞淋巴瘤(DLBCL)中采用免疫组织化学EnVision法检测CD44、TCLl以及CD10、bel-2、bcl-6、c-myc、Ki-67等常用抗体表达情况.结果 Burkitt淋巴瘤中瘤细胞96%(24例)呈TCL1阳性,仅4%(1例)CD44阳性;88%(22例)CD10阳性、92%(23例)bcl-6和c-myc阳性,仅4%(1例)bcl-2阳性;Ki-67增殖指数为95%～100%.非特指DLBCL中仅16%(4例)TCL1弱阳性,84%(21例)CD44阳性、32%(8例)CD10阳性、72%(18例)bcl-6和bcl-2阳性、c-myc均阴性,Ki-67增殖指数40%～90%.结论 当形态和免疫表型不典型时,TCL1和CD44两种蛋白的检测有助于提高Burkitt淋巴瘤的确诊率及其与DLBCL的鉴别诊断.     

ALK阳性的弥漫性大B细胞淋巴瘤

目的 探讨间变性淋巴瘤激酶(ALK)阳性的弥漫性大B细胞淋巴瘤的组织病理形态和免疫组化表达的意义。方法 参照WHO2001年恶性淋巴瘤分类,对222例弥漫性大B细胞淋巴瘤进行形态学观察和免疫组化Polymer两步法标记。结果 6例弥漫性大B细胞淋巴瘤免疫组化ALK阳性表达,阳性反应物质定位于细胞质内,成粗大的颗粒状,1例合并CD30阳性表达,全部表达B系列抗原CD20、CD79α和CD138,4例不表达CD45。组织病理形态：3例为浆母细胞性,2例为免疫母细胞性伴浆细胞样分化,1例为间变性。结论 ALK阳性的弥漫性大B细胞淋巴瘤是组织形态和免疫表型独特的变异类型,与CLTC—ALK基因易位和NPM—ALK融合基因易位有关,其分子遗传学的异质性不同于以往的认识。     

原发性皮肤间变性大细胞淋巴瘤临床病理分析

目的 探讨原发性皮肤间变性大细胞淋巴瘤(C-ALCL)的临床病理特征、免疫表型及预后.方法 分析8例C-ALCL的临床病理资料,复习HE切片,进行T淋巴细胞、B淋巴细胞、活化淋巴细胞和细胞毒性等16种标记的免疫组织化学染色,原位杂交检测EB病毒.结果 8例中男3例,女5例,中位年龄49.5岁.临床上以皮肤无症状的单个红色结节、肿块为主要表现,组织学上肿瘤细胞在真皮与皮下脂肪内大片状、弥漫性浸润.瘤细胞以大细胞为主,异形性明显.8例C-ALCL的瘤细胞CD30阳性细胞数均大于75%.瘤细胞均表达1～3个T细胞标记(CD3、CD5或CD45RO)及1～3个细胞毒性标记[T细胞内抗原(TIA)-1、颗粒酶B或穿孔素].表达白细胞共同抗原(LCA)为8例、CIM为5例、CD8为1例、间变性淋巴瘤激酶(ALK)-1为1例、上皮细胞膜抗原(EMA)为3例,均不表达CD15、CD20、CK和HMB45.EBER 1/2原位杂交均为阴性.获随访的6例中5例存活,1例死亡(死因不详).结论 C-ALCL有独特的临床病理表现和免疫表型,预后较好.EB病毒与C-ALCL可能无明确的相关性.     

胃肠道B细胞性淋巴瘤形态学及免疫组织化学分析

目的 探讨胃肠道B细胞淋巴瘤的分类特点及病理诊断.方法 对194例胃肠道B细胞淋巴瘤分别进行HE染色和免疫组织化学染色,临床病理学观察内容包括:患者性别、年龄、肿瘤发生部位、浸润深度、组织结构(淋巴上皮病变、反应性/残留淋巴滤泡、凝固性坏死/坏死碎片、结节状生长方式).免疫组织化学染色采用EnVision二步法,每例标记9种抗体,包括:Pan B、Pan T、bcl-6、CD10、bcl-10、cyclin D1,末端脱氧核苷酸转移酶(TdT)、MUM1、Ki-67.结果 194例胃肠道B细胞淋巴瘤的男女之比为1.4∶1;发病年龄最小为8岁,最大为85岁.诊断为弥漫性大B细胞淋巴瘤(DLBBCL)128例(66.0%),其中DLBCL伴黏膜相关淋巴组织边缘区B细胞淋巴瘤(MALT淋巴瘤)成分的有16例;MALT淋巴瘤40例(20.6%);滤泡性淋巴瘤(FL)8例(4.1%);淋巴浆细胞性淋巴瘤(LPL)5例(2.6%);套细胞淋巴瘤(MCL)3例(1.6%);B淋巴母细胞性淋巴瘤(B-LBL)1例(0.5%);不能分型9例(4.6%,其中5例为活检标本).发生于胃100例(51.5%)、小肠43例(22.2%)、回盲部26例(13.4%)、阑尾1例(0.5%)、结肠21例(10.8%)、直肠3例(1.6%).163例手术切除标本中侵犯黏膜层20例(12.3%)、浅肌层20例(12.3%)、深肌层19例(11.6%)、全层104例(63.8%).见有淋巴上皮病变、反应性/残留淋巴滤泡、凝固性坏死/坏死碎片、结节状生长改变者分别为52、29、66和30例.免疫组织化学标记,194例均表达CD20而不表达CD3,不同类型的淋巴瘤对bcl-6、CD10、bcl-10、cycin D1、TdT、MUM1、Ki-67有不同程度的表达.结论 胃肠道B细胞淋巴瘤主要分大B细胞性和小B细胞性两大类,小B细胞性淋巴瘤的分型是病理诊断的难点.对胃肠道B细胞淋巴瘤的诊断方法提出了建议路线.     

原发性中枢神经系统弥漫性大B细胞淋巴瘤主要为活化B细胞亚型

目的 对原发性中枢神经系统弥漫性大B细胞淋巴瘤分型,并探讨其组织起源和预后相关意义.方法 应用免疫组织化学EnVision二步法,检测CD10、bcl-6、MUM-1、CD138和FOXP1在47例原发性中枢神经系统弥漫性大B细胞淋巴瘤中的表达情况.结果 CD10、bcl-6、MUM-1、CD138和FOXPI表达率分别为6.4%、53.2%、91.5%、0和93.6%.47例中有43例(91.5%)为活化B细胞表型:21例(44.7%)为活化的生发中心亚型,22例(46.8%)为活化的非生发中心亚型.该分型及FOXP1的表达与预后无明显相关性(P=0.279和P=0.154).结论 原发性中枢神经系统弥漫性大B细胞淋巴瘤绝大多数为活化B细胞亚型,是系统性弥漫性大B细胞淋巴瘤中一种相对同质性的亚型,推测其组织起源是生发中心末期至后生发中心早期的B细胞.     

胃T细胞淋巴瘤临床病理观察

目的 探讨胃T细胞淋巴瘤的临床病理学特点.方法 收集7例胃T细胞淋巴瘤病例标本,对其进行了临床病理分析、免疫组织化学检测、EBER原位杂交检测及T细胞受体(TCR)基因重排检测.结果 7例病例中6例为男性,1例为女性,平均发病年龄为45岁.6例可获得资料的病例中,1例有长期腹泻史,5例有低蛋白血症.组织学上,7例标本中,有5例表现为肿瘤细胞体积较大而不一致,2例表现为大小一致的中等细胞.1例病例可见肿瘤细胞浸润腺上皮.所有病例的肿瘤组织均不表达CD20及CD79a.7例病例中,各有6例表达CD3及T细胞胞质内抗原,各有4例表达CD5、βF-1及CD30,有3例表达CDM,各有1例病例表达CD8、CD56、问变性淋巴瘤激酶及粒酶B.7例病例肿瘤细胞EBER原位杂交检测均为阴性且都存在TCR基因克隆性重排.结论 胃T细胞淋巴瘤是一种少见的恶性淋巴瘤,具有独特的临床病理特点.     

原发淋巴结套细胞淋巴瘤临床病理分析

目的：探讨原发淋巴结套细胞淋巴瘤（MCL）的临床病理与免疫组化特点。方法：收集6例淋巴结MCL,免疫组化ABC法确定肿瘤细胞特征,使用的抗体有CD45、CD20、CD79、CD45RO、CD30、CD68、TdT、CD43、CD5、cyclinD1、c-myc,IgD,IgM等。结果：光镜可将MCL分为4种亚型：套区型1例,结节型1例,弥漫型2例,母细胞化型2例。肿瘤细胞表达全B细胞标记,IgD CD43 ,cyclinD1(5/6),CD5(4/6) 。结论：MCL是一种具有特殊免疫表型的B细胞淋巴瘤,不同的组织学构型其预后可能不同,临床应与其它类型B细胞淋巴瘤鉴别,如淋巴结边缘区B细胞淋巴瘤（MZL）,滤泡性淋巴瘤（FL）及CLL／SLL等鉴别。     

儿童及青少年原发性中枢神经系统T细胞淋巴瘤5例临床病理学分析

目的探讨儿童及青少年原发性中枢神经系统T细胞淋巴瘤(PCNSTCL)的临床病理特征, 以提高对本病的认识。方法收集郑州大学第一附属医院2016年12月至2021年12月数据库里确诊的儿童及青少年PCNSTCL病例5例, 对其临床特征、病理学特征、免疫表型及分子病理学特征进行观察, 并复习相关文献。结果 5例患者中, 男性2例, 女性3例, 年龄范围在11～18岁, 中位年龄14岁。2例诊断为非特指型外周T细胞淋巴瘤, 2例诊断为间变性淋巴瘤激酶(ALK)阳性的间变性大细胞淋巴瘤, 1例诊断为NK/T细胞淋巴瘤。病理学特征:肿瘤细胞弥漫性生长, 组织学形态谱系较广, 可以是小、中、大的细胞, 局部可见肿瘤细胞围绕血管壁生长。免疫组织化学及原位杂交:4例表达CD3, 1例表达缺失;4例CD5表达缺失, 1例未缺失;2例同时表达ALK及CD30;1例表达CD56及EB病毒编码的RNA阳性;5例均表达细胞毒性标志物T细胞胞质内抗原1及颗粒酶B;3例Ki-67阳性指数>50%。2例进行T细胞受体(TCR)基因重排检测, 显示单克隆性增生。结论 PCNSTCL较为少见, 发生于儿童及青少年的...     

原发于骨骼肌的间变性大细胞T细胞淋巴瘤

目的：探讨骨骼肌原发的间变性大细胞淋巴瘤的临床病理特征和免疫表型。方法：采用常规制片和免疫组化(S-P)法检测1例（14岁）骨骼肌原发的间变性大细胞淋巴瘤。结果：肿瘤细胞CD30、ALK-1、CD45RO和CD45阳性；而CD20、EMA、S-100蛋白、desmin和CD68阴性。结论：本例为间变性淋巴瘤激酶（ALK）阳性的间变性大细胞淋巴瘤。骨骼肌原发的间变性大细胞淋巴瘤非常少见，诊断旱应先排除其它肿瘤和其它部位淋巴瘤累及骨骼肌。     

淋巴母细胞性淋巴瘤/白血病26例细胞病理学分析

目的探讨利用细胞学诊断淋巴母细胞性淋巴瘤/白血病的可行性和准确性。方法选择由细胞学初诊、淋巴结或骨髓活检确诊的淋巴母细胞性淋巴瘤/白血病26例,包括浅表淋巴结细针穿刺13例,纵隔包块细针穿刺1例,胸腔积液9例,心包积液1例,脑脊液2例,对所有病例的临床特点、细胞形态和免疫细胞化学进行分析。结果所有病例均查见较多中等偏小的淋巴样肿瘤细胞,其免疫表型为22例(84·6%)表达TdT,22例(84·6%)表达CD99,23例(88·5%)表达CD3ε,3例(11·5%)表达CD20,26例均不表达MPO,Ki-67PI均值为78·6%。结论观察细胞涂片中细胞的形态可以对淋巴母细胞性淋巴瘤/白血病进行初步诊断,结合临床表现和免疫细胞化学结果可进一步提高诊断的准确性和可靠性。     

Renal dysplasia and asplenia in two sibs

A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.
This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.     

Schizophrenia in a North Swedish geographical isolate, 1900–1977. Epidemiology, genetics and biochemistry

Over 200 schizophrenic patients belonging to three major and interrelated pedigree complexes have been investigated over the past 30 years in a North Swedish geographically isolated population, presently numbering about 6,000. An intensive investigation of a number of biochemical correlates and genetic markers in a few selected families belonging to one of the major pedigrees has indicated new strategies for the current research program.
Schizophrenia, as defined operationally, is significantly associated with decreased activities of two enzymes (1) blood platelet monoamine oxidase, (2) plasma dopamine-β-hydroxylase, and (3) with the genetic marker Gc² (group specific antigen). Both enzymes are subject to genetic variation. A positive score for linkage between schizophrenia and low plasma DBH activity has been calculated, but, so far, available data are insufficient for discrimination between linkage and partial contribution of genetically controlled low plasma DBH to the pathogenesis of the disease. Alternatively, both mechanisms could be involved.
As a model for continued research, schizophrenia is explained as based on a double dominant-recessive genotype (Aabb), representing a vulnerability which in about 50 % of cases develops into clinical schizophrenia. It is suggested that the dominant mutation (A) operates on or affects MAO activity, and that the recessive genotype (bb) is instrumental in low variates of DBH activity and very likely such variates within the normal range of physiological variation. Moreover, it is suggested that the combined effects of MAO- and DBH-reduced efficiency on the metabolism of e.g. dopamine could be an essential pathogenic mechanism for the schizophrenic illness which is segregating in this population.     

The dominant diseases of modernized societies as omega-3 essential fatty acid deficiency syndrome: Substrate beriberi

About 1900, modern food selection and processing caused widespread $\text{epidemics}$ of the B vitamin deficiency diseases of beriberi and pellagra which, for genetic reasons, often expressed as different diseases ranging from bowel and heart disease to dermatoses and psychoses. But the B vitamins merely help convert essential fatty acids (EFA) into the prostaglandin (PG) tissue regulators and it now turns out that, through hydrogenation, milling and selection of w3-poor southern foods, we have also been systematically depleting, by as much as 90%, a newly discovered trace Nordic EFA (w3) of special importance to primates and sole precursor of the PG3(4) series, even as a concurrent fiber deficiency increases body demand for EFA. Since substrate EFA is processed by many B vitamin catalysts, an EFA deficiency will mimic a $\text{panh}$ypovitaminosis B, i.e., a mixture of $\text{substrate}$ beriberi and $\text{substrate}$ pellagra resembling vitamin beriberi and pellagra but exhibiting as even more diverse $\text{endemic}$ disease. This would consitute a second stage of the Modern Malnutrition and explain why some workers now hold the dominant diseases of modermized societies to be new, nutritionally based, pellagraform yet lipid-related and to range, once again, from heart disease to psychosis. It is an assumption that our dominant diseases are unrelated to each other or are merely revealed by our diagnostic acumen and therapeutic success; and that hydrogenating millions of tons of food oils annually, to destroy the rancidity producing w3-EFA, is safe for $\text{primates}$. Extensive beriberiform disease is reported here in 32 typical cases taken from medical practice which responds strikingly to linseed oil supplements (60% w3-EFA) in confirmation of identical results in Capuchins.     

What will studies of Fulani individuals naturally exposed to malaria teach us about protective immunity to malaria?

There are an estimated over 200 million yearly cases of malaria worldwide. Despite concerted international effort to combat the disease, it still causes approximately half a million deaths every year, the majority of which are young children with Plasmodium falciparum infection in sub-Saharan Africa. Successes are largely attributed to malaria prevention strategies, such as insecticide-treated mosquito nets and indoor spraying, as well as improved access to existing treatments. One important hurdle to new approaches for the treatment and prevention of malaria is our limited understanding of the biology of Plasmodium infection and its complex interaction with the immune system of its human host. Therefore, the elimination of malaria in Africa not only relies on existing tools to reduce malaria burden, but also requires fundamental research to develop innovative approaches. Here, we summarize our discoveries from investigations of ethnic groups of West Africa who have different susceptibility to malaria.     

'Very sore nights and days': the child's experience of illness in early modern England, c.1580-1720

Sick children were ubiquitous in early modern England, and yet they have received very little attention from historians. Taking the elusive perspective of the child, this article explores the physical, emotional, and spiritual experience of illness in England between approximately 1580 and 1720. What was it like being ill and suffering pain? How did the young respond emotionally to the anticipation of death? It is argued that children’s experiences were characterised by profound ambivalence: illness could be terrifying and distressing, but also a source of emotional and spiritual fulfilment and joy. This interpretation challenges the common assumption amongst medical historians that the experiences of early modern patients were utterly miserable. It also sheds light on children’s emotional feelings for their parents, a subject often overlooked in the historiography of childhood. The primary sources used in this article include diaries, autobiographies, letters, the biographies of pious children, printed possession cases, doctors’ casebooks, and theological treatises concerning the afterlife.     

Guidelines for the Validation and Use of Immunoassays for Determination of Introduced Proteins in Biotechnology Enhanced Crops and Derived Food Ingredients

Recent advancements in agricultural biotechnology have created a need for analytical techniques to determine introduced proteins in crops enhanced through modern biotechnology techniques. These proteins are expressed in plant tissues and may be present in food ingredients. Immunoassays are ideally suited for protein detection and may be used as both quantitative and threshold methods. Microplate ELISA and lateral flow devices are two of the most commonly used immunoassay formats for agricultural biotechnology applications. This paper provides general background information and a discussion of criteria for the validation and application of immunochemical methods to the analysis of proteins introduced into plants and food ingredients using biotechnology methods. It is the result of a collaborative effort of members of the Analytical Environmental Immunochemical Consortium. This collaborative effort represents the combined expertise of several organizations to reach consensus on establishing guidelines for the validation and use of immunoassays. Further, the paper offers developers and users a consistent approach to adopting the technology as well as aid in producing accurate and meaningful results.     

HLA in North Colombia Chimila Amerindians

HLA-A,-B,-C,-DRB1 and -DQB1 alleles have been studied in Chimila Amerindians from Sabana de San Angel (North Colombian Coast) by using high resolution molecular typing. A frequent extended haplotype was found:HLA-A*24:02-B*51:10-C*15:02-BRB1*04:07-DQB1*03:02 (28.7%) which has also been described in Amerinndian Mayos Mexican population (Mexico, California Gulf, Pacific Ocean). Other haplotypes had already been found in Amerindians from Mexico (Pacific and Atlantic Coast), Peru (highlands and Amazon Basin), Bolivia and North USA. A geographic pattern according to HLA allele or haplotype frequencies is lacking in Amerindians, as already known. Also, five new extended haplotypes were found in Chimila Amerindians. Their HLA-A*24:02 high frequencies characteristic is shared with aboriginal populations of Taiwan; also, HLA-C*01:02 high frequencies are found in New Zealand Maoris, New Caledonians and Kimberly Aborigines from Australia. Finally, this study may show a model of evolutionary factors acting and rising one HLA allele frequency (-A*24:02), but not in others that belong to the same or different HLA loci.     

Ultracryotomy: development and application (with examples from the yeast cytology) (author's transl)

The preparation steps usually necessary for obtaining ultrathin frozen sections of biological material (chemical prefixation, enclosing, cryoprotective treatment, freezing, sectioning, and post-staining the sections for transmission electron microscopy) are submitted to a critical analysis. The application of cryo-ultramicrotomy, in particularly for cytochemical purposes, is reviewed. Fundamental considerations of chemical prefixation and poststaining are supported by examples from yeast cytology. Furthermore, the efficiency of the cryo-ultramicrotomy (electron optical resolution of ultrastructural details) is demonstrated on yeast cells and protoplasts.     

The universal muscular chamber. A basic unit of the genitourinary, cardiovascular, gastro-intestinal, skeletal, cutaneous, respiratory and other systems, endocameral hypertension; and spasm, the key to their idiopathic diseases and arthropathies

Starting with the integument, we see many organs are contractile sacs or multiples thereof, which tubes or bags constitute the major part of the entire body. Recognition of this basic unit and its characteristics sheds new light, individually and collectively, on many disorders previously considered unrelated. Muscular tears and perforations develop in the walls of these chambers, being no way peculiar to those organs, wherein, hydrochloric acid occurs. So, it is not necessary to explain the absence of excessive acid from patients who exhibit holes in the gastric, uterine, aortic, duodenal, rectal, pulmonary, retina, and other walls. Muscle, not acid is the great common factor relating idiopathic disorders in the gastrointestinal tract to each other and to similar diseases in other systems. When the units are linked together, the lesions tend to appear as arthropathies, i.e. at the joints. Rephrasing common-place observations, frees us from conventional, conceptual cul-de-sacs. An observation is only as good as its interpretation, so all possibilities must be considered, otherwise, we will remain blinded by our misconceptions.