Ph染色体阳性急性髓系白血病细胞免疫表型及临床特征研究

目的　探讨 Ph染色体阳性急性髓系白血病 ( AML)患者细胞免疫表型及临床特征。方法　对 7例初诊为 Ph染色体阳性 AML 患者进行细胞形态学、细胞遗传学及细胞免疫表型检测 ,结合患者白细胞数、感染情况及临床治疗的疗效和生存期进行综合分析。结果　 Ph染色体阳性 AML7例 ,占同期 AML 患者的 3 .5 % ,细胞免疫表型伴淋系表达率高 (占 4 2 .9% ) ,CD3 4表达均阳性。完全缓解率为 4 2 .9% ,平均生存期 11个月。结论　 Ph染色体阳性 AML在细胞免疫表型、形态学及临床特征方面有其特殊性 ,疗效差 ,生存期短     

Immunohistochemical profile of lymphoid lesions of the orbit

BACKGROUND: Orbital idiopathic inflammation, lymphoid hyperplasia, and lymphoma may all present clinically in the same manner. Histopathology and especially immunohistochemistry play a major role in the differential diagnosis. The purpose of this study was to determine the immunophenotypic features of these lesions. METHODS: Fifty-five orbital lymphoid lesions were retrieved from the ophthalmic pathology registries at McGill University, Montreal, Canada, and the Federal University of S?o Paulo, S?o Paulo, Brazil. Formalin-fixed, paraffin-embedded, histopathologic sections were stained with hematoxylin and eosin and periodic acid-Schiff. The sections were also immunostained for B-cell (CD20) and T-cell (CD43) markers and for immunoglobulin light chains kappa and lambda. Two pathologists determined the histopathologic and immunohistochemical pattern of each lesion in a masked fashion. RESULTS: Of the 55 lesions, 11 (20%) were idiopathic chronic inflammations, 22 (40%) were lymphoid hyperplasias and 22 (40%) were lymphomas. Idiopathic inflammation displayed a predominance of T cells and all lesions expressed polyclonal light chains. Lymphoid hyperplasia displayed a mixture of B cells and T cells, with a slight predominance of the former and all lesions expressed polyclonal light chains. Lymphoma showed a striking predominance of B cells and all lesions expressed monoclonal light chains, usually kappa (63.7%). The differences in the mean percentages of B cells among the orbital lymphoid lesions (inflammation, 35%; hyperplasia, 65.9%; lymphoma, 87.3%) were statistically significant (p < 0.001). INTERPRETATION: Orbital lymphoid lesions can be differentiated based on the percentages of B cells and T cells and the monoclonal or polyclonal expression of immunoglobulin light chains.     

急性淋巴细胞白血病伴CD13表达免疫表型分析

目的：探讨急性淋巴细胞白血病（acutelymphocytic leukemia,ALL）伴CD13表达的免疫表型特点,对包括CD33在内的其他抗原进行相关性分析.方法：对85例初诊ALL患者进行免疫学分型,以是否表达My把B-ALL和T-ALL分为My＋ALL和My-ALL,进行组间分析.结果：全部85例B-ALL和T-ALL患者均高表达B系和T系相关抗原（100%,100%）.所有的B-ALL均不表达T-ALL相关抗原,所有T-ALL均不表达B-ALL相关抗原.CD13的表达率为31%（B-ALL31.4%,T-ALL 28.6%）.My＋B-ALL和My＋T-ALL患者的CD13阳性细胞的中位数高于My-B-ALL和My-T-ALL患者,P值分别为0.013和0.04.My＋B-ALL患者的CD15阳性细胞的中位数高于My-B-ALL患者,P=0.000 1.结论：白血病免疫分型对于白血病的诊断、治疗和预后判断均有很大帮助,其在临床诊断中的推广将有助于患者的诊断和治疗.CD13在My＋ALL中表达较高（31%）,白血病细胞在恶性演变过程中不同时期表现出特征反映,其临床意义有待于重新评价.     

Progressive form of pulmonary Langenharns' cell histiocytosis in a female adult non-smoker

Little is known about the cause, nature, treatment and prognosis of pulmonary Langerhans' cell histiocytosis (LCH) in adults. We report the case of a 44-year-old female non-smoker suffering from pulmonary histiocytosis who after a 7-year remission period relapsed with both lung and bone disease. Using a combination of corticosteroids, methotrexate and bone irradiation treatment, the patient achieved total disease remission. The patient was a non-smoking female who has had long-term and swift remission of the disease on two occasions.     

三色流式细胞术分析儿童初治急性淋巴细胞白血病免疫表型特征

目的:应用三色流式细胞术研究儿童急性淋巴细胞白血病(AL L)免疫表型特征。方法:采用CD4 5/ SSC双参数散点图设门方法检测37例儿童AL L 免疫表型。结果:37例儿童AL L 中B- AL L 34例,占91.9% ,其中CD1 9阳性表达率10 0 % ,明显高于CD1 0 (82 .4 % ,P<0 .0 5 )和CD2 2 (6 1.8% ,P<0 .0 1)的表达。T- AL L 2例,仅占5 .4 % ;CD34 /HL A- DR阳性率较高(86 .5 % / 81.1% ) ,二者表达较一致;AL L 伴髓系抗原表达占2 7% ,主要表达CD33。结论:儿童AL L中以B- AL L为主,B- AL L白血病细胞稳定持续表达CD1 9,CD1 9是诊断B- AL L较为可靠的表面标记。此外,儿童AL L具有较高的CD34 和髓系抗原表达。     

Granulomatous inflammation in Acanthamoeba keratitis: an immunohistochemical study of five cases and review of literature

Purpose: Acanthamoeba keratitis usually presents as a necrotizing stromal inflammation. We report a rare presentation of granulomatous inflammation in Acanthamoeba keratitis Methods: Retrospective clinico-pathologic case series. Results: Five corneal tissues (3 corneal buttons, 2-eviscerated contents) from patients suffering from severe Acanthamoeba keratitis not responding to anti-Acanthamoeba treatment, revealed a florid granulomotous inflammation with multinucleated giant cells in the posterior stroma and around Descemet’s membrane. Phagocytosed parasites were noted within the giant cells. Vascularization of the corneal stroma was noted in two cases. Immunophenotyping revealed a predominance of T lymphocytes and macrophages. Clinically, four of five cases had shown features of limbal and scleral involvement. Conclusion: Granulomatous inflammation in the posterior corneal stroma, is not an uncommon finding in Acanthamoeba keratitis and could possibly be immune-mediated, contributing to persistence and progression of disease. Clinical Relevance: Presence of granulomatous inflammation in Acanthamoeba keratitis, in most cases is associated with limbal and scleral involvement and therefore could be considered as one of the poor prognostic markers. Further studies are required to ascertain the specific clinical features and appropriate management strategies in these cases.     

The role of flow cytometric immunophenotyping in improving the diagnostic accuracy in referred fine-needle aspiration specimens

Flow cytometric (FCM) immunophenotyping has an important role in the diagnostic work up of fine-needle aspiration (FNA) specimens obtained from lymphoid lesions. The objective of the present study was to evaluate the feasibility of this method with respect to referred FNA specimens. One hundred and two FNA specimens referred to our laboratory for FCM analysis during the last 3 years were studied. Specimens were sent, accompanied by cytological smears, from 11 distant hospitals by ordinary mail. The evaluation of potential B-cell monoclonality, the main diagnostic issue to be resolved using FCM, was possible in 86 of these 102 cases. The remaining 16 samples could not be analyzed or adequately interpreted because of sparse or necrotic material. A monoclonal B-cell population was found in 17/86 satisfactory cases, of which 16 were histologically confirmed. Eight cases contained cells positive for the epithelial marker Ber-EP4 and were diagnosed accordingly as carcinomas. FCM analysis of specimens obtained with a clinical question of Hodgkin lymphoma or T-cell lymphomas did not yield definitive data. The time lapse between sampling and analysis (12-84 hr) did not affect the results. This probably was due to the fact that all aspirates were taken in Roswell Park Memorial Institute (RPMI) cell medium, supplemented with 50% fetal calf serum. In conclusion, this retrospective study establishes that it is possible, in the majority of cases, to refer FNA material for FCM immunophenotyping by mail, and that results regarding B-cell clonality in the case of small-cell lymphomas are reliable also after a transportation period of 3-4 days. Carcinoma may be similarly diagnosed and a diagnosis of lymphoma may be excluded in reactive proliferations. Cases with only a few atypical cells or specimens from patients suspected of having Hodgkin lymphoma or T-cell lymphomas are not suitable for analysis by FCM.     

Differences in peripheral blood lymphocyte phenotypes between Helicobacter pylori-positive children and adults with duodenal ulcer

The immunological mechanisms involved in the development of duodenal ulcer, especially in childhood, are unclear. Helicobacter pylori-positive children and adults, with and without duodenal ulcer, were therefore compared with respect to CD4(+) T-cells, and CD8(+) T-cells, B-cells and B1a-cells, as well as cell activation (CD4(+)/HLA-DR(+) and CD8(+)/HLA-DR(+)) and co-stimulatory (CD4(+)/CD28(+) and CD8(+)/CD28(+)) markers, in peripheral blood. Children with and without duodenal ulcer differed significantly. In particular, there was a phenotypic change in CD8(+) T-cells from children with ulcer that involved a 200% increase in the number of CD8(+)/HLA-DR(+) cells/mm(3) and a decrease of 34.2% in the number of CD8(+)/CD28(+) cells/mm(3). This phenotype of chronically activated memory CD8(+) T-cells, which has also been observed in patients with AIDS and tuberculosis, is associated with disease severity and progression. A lower frequency of B1a-cells was also observed in the group of children with ulcer. Conversely, no difference between infected adults with and without ulcer was observed, but the percentage of CD4(+)/HLA-DR(+) cells was lower in adults with ulcer, suggesting that a down-regulated immune response may play a role in the development of duodenal ulcer in adults. Gastric inflammation correlated positively with CD4(+) and chronically activated CD4(+) T-cells in children and adults without duodenal ulcer, respectively. These results suggest that there are differences in the immunophenotyping profile between H. pylori-positive children and adults with duodenal ulcer, indicating the possibility of distinct immune mechanisms in the development of the disease according to age.     

流式细胞术对骨髓增生异常综合征和再生障碍性贫血患者骨髓细胞免疫表型的分析

检测骨髓增生异常综合征(MDS)和再生障碍性贫血(AA)患者骨髓细胞免疫表型特征,探讨其对二者发病机制、分型诊断及鉴别诊断的临床意义。选用多种单克隆抗体,应用直接免疫荧光法采用流式细胞术CD45/SSC设门,对23例MDS、14例AA、9例正常对照的骨髓细胞各免疫标志的表达率进行检测。结果:MDS组与正常对照组相比其造血干/祖细胞抗原CD34、HLA-DR、髓系早期抗原CD13、CD33、单核系抗原CD14、T淋巴细胞抗原CD7的表达率增高,髓系成熟抗原CD15、B淋巴系抗原CD19、CD20的表达率降低,DI值增高;由RA进展至RAEB,CD34、HLA-DR、CD13、CD33、CD14、CD7的表达率逐渐增高,CD15、CD3、CD19、CD20的表达率逐渐减低,DI值逐渐增高。AA组与正常对照组相比其CD34、CD13、CD33、CD15的表达率降低,CD3、CD7、CD25、CD22的表达率增高。MDS组与AA组相比其CD34、HLA-DR、CD13、CD33、CD14的表达率增高,CD3、CD5、CD7、CD15、CD19、CD20、CD22、CD25的表达率显著降低。MDS和AA患者骨髓细胞免疫表型分析,有助于揭示二者的发病机制,为临床提供新的诊断、分型及鉴别诊断方法。     

ADVANCES IN THE UNDERSTANDING OF THE MOLECULAR PATHOLOGY OF CONNECTIVE TISSUE NEOPLASMS

Although primary malignant neoplasms of the connective tissues are uncommon, they have been subjected to intense study by modern molecular and other analytical techniques. Aberrations of genes and their products are providing insights into aetiopathogenesis and indicating improved methods of histopathological diagnosis and patient management. © 1997 by John Wiley & Sons, Ltd.