Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.11 (CD10)
 9,792 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The object of this study was to evaluate the treatment outcome in children with acute lymphoblastic leukemia (ALL) in Chennai. The problems inherent in a developing country which affect outcome are analyzed. The importance of prognostic factors especially immunotyping is assessed. The period of study was from June 1991 to December 1995. A total of 135 children were studied. Pre B CALLA positive (CD10, CD19, HLA, DR) was the dominant immunotype in 75 children (69 per cent). T-cell ALL was seen in 15 (14 per cent), biphenotype in three (2 per cent), and B in one (0.9 per cent). Seventy children (53 per cent) were treated with a high risk protocol, 25 (17 per cent) received an intermediate risk, and 40 patients (30 per cent) received a standard risk protocol. Analyzing the outcome in 135 children, 34 (27 per cent) had event free survival (EFS) at the time of analysis; of these 41 per cent had EFS after 2 years of therapy, 31 per cent after 3 years and 18.7 per cent after 4 years (i.e. 1 year after stopping 3 years of therapy). Fifty-seven children (41 per cent) dropped out; 25 (18 per cent) died due to sepsis. Treatment obstacles included delay in diagnosis, poor health education and facilities, poor supportive care, and socio-economic problems.
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PMID:Survival of childhood acute lymphoblastic leukemia: experience in Chennai. 1066 9

Two 4-year-old monozygotic Chinese, female twins developed concordant childhood acute lymphoblastic leukemia (ALL) within an interval of about 2 weeks. Based on morphology and cytochemistry findings of the bone marrow blast cells, a diagnosis of ALL, L1 was made. Immunophenotyping showed the blast cells of both twins expressed similar antigens, i.e. HLA-DR, CD10, CD13, CD19, CD22 and CD34. Identical blood group, same HLA (human leucocyte antigen) genotype, sex and similar appearance suggest that the twins are monozygotic. Since the bone marrow leukemic cells of both twins were identical in morphology and expressed the same antigens with almost similar percentages of positivity, it is likely that the blast cells were derived from the same single clone. Based on the single clone hypothesis, the leukemogenic event must have arisen in utero in one twin and the cells from the abnormal clone then spread to the other twin via shared placental anastomoses.
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PMID:Concordant childhood acute lymphoblastic leukemia in monozygotic twins. 1096 97

Primary mediastinal B-cell lymphoma is a locally highly aggressive but poorly disseminating tumor composed of medium sized or large cells most probably of thymic medullary origin. It has a mature B-cell phenotype, typically lacks immunoglobulin expression and has variable defects in expression of HLA-molecules. We present here a cell line, MedB-1, derived from such a tumor. As is frequently found in mediastinal B-cell lymphomas in situ, MedB-1 is CD10(-), CD19(+), CD21(-), CD22(+), CD23(+), CD25(-), CD37(+), CD38(-), CD39(+), CD40(+), CD54(+), CD95(+). Like the parental tumor, MedB-1 lacks HLA-A,B,C alpha-chains and beta(2)microglobulin and expresses HLA-D molecules at decreased levels. Both parental tumor and MedB-1 cells are clonally related as shown by immunoglobulin heavy chain gene rearrangement analysis. Unlike the parental tumor tissue, the MedB-1 cell line cytoplasmically expresses IgG/kappa in a very small subset of cells under standard culture conditions. MedB-1 does not contain any Epstein-Barr virus DNA. In a tissue adhesion assay MedB-1 cells showed an extensive binding to the medullary region of normal thymus. Altogether, MedB-1 is a suitable tool for functional and molecular analysis of this distinct lymphoma entity.
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PMID:MedB-1, a human tumor cell line derived from a primary mediastinal large B-cell lymphoma. 1129 Oct 70

Little is known about the processing of putative human autoantigens and why tolerance is established to some T cell epitopes but not others. Here we show that a principal human HLA-DR2-restricted epitope--amino acids 85-99 of myelin basic protein, MBP(85-99)--contains a processing site for the cysteine protease asparagine endopeptidase (AEP). Presentation of this epitope by human antigen-presenting cells is inversely proportional to the amount of cellular AEP activity: inhibition of AEP in living cells greatly enhances presentation of the MBP(85-99) epitope, whereas overexpression of AEP diminishes presentation. These results indicate that central tolerance to this encephalitogenic MBP epitope may not be established because destructive processing limits its display in the thymus. Consistent with this hypothesis, AEP is expressed abundantly in thymic antigen-presenting cells.
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PMID:Destructive processing by asparagine endopeptidase limits presentation of a dominant T cell epitope in MBP. 1181 83

Caco-2 is a colonic tumour cell line which, when cultured, spontaneously exhibits enterocyte-like characteristics. Given the difficulties in maintaining long-lasting cultures of enterocytes, this cell line may be a suitable in vitro model to carry out experiments trying to delineate the involvement of enterocytes in local immune responses, and their role in pathology. It seems then reasonable to obtain a detailed immune analysis of Caco-2, and compare it with available data on enterocytes. Cytofluorometry revealed several leukocyte markers on Caco-2, present also on human enterocytes. These markers include surface proteases (CD10, CD13 and CD26), antigen-presenting cell markers (CD13, CD14, CD35 and CD63), integrins (CD18 and CD61), epithelial/endothelial markers (CD21, CD31, CD47 and CD59) and finally, CD25 and CD28. In contrast to enterocytes, HLA-class 11 molecules are not found on Caco-2, whether resting or gamma-IFN-stimulated. Moreover, culture experiments with allogeneic lymphocytes revealed that Caco-2 cells were unable to induce their proliferation. Cytokine analysis showed an increased RANTES synthesis and IL-2 transcription upon stimulation with IL-1beta. Finally, amongst RANTES receptors, CCR1 is found on Caco-2 cells, whereas CCR3 and CCR5 are not.
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PMID:Cell surface phenotype and cytokine secretion in Caco-2 cell cultures: increased RANTES production and IL-2 transcription upon stimulation with IL-1beta. 1182 1

We describe the case of a 23-year-old man with acute lymphoblastic leukemia in whom the Philadelphia chromosome was first detected in the late stage of the disease. At diagnosis, the patient's leukocyte count was 39,400/microliter and leukemic cells were positive for CD10, 19, 20, 33, 34 and HLA-DR. Karyotypic analysis at diagnosis revealed 46,XY. Complete remission was achieved after the first induction therapy, but the disease recurred after 9 months. The patient underwent allogeneic peripheral blood stem cell transplantation from his HLA identical mother, but relapse occurred on day 80. The proportion of bone marrow lymphoblasts decreased transiently after donor lymphocyte infusion but later increased, and the patient died on day 362. The Philadelphia chromosome was first detected by karyotypic analysis on day 256. p190-type bcr/abl mRNA transcripts were negative following RT-PCR at the initial diagnosis, but became positive from the first relapse through the late stage. Generally, the product of the bcr/abl fusion gene has been thought to play an important role in leukemogenesis, however the present case suggests that this gene product is also related to disease progression.
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PMID:[Acute lymphoblastic leukemia with p190 type bcr/abl chimeric mRNA at relapse]. 1241 88

Aggressive natural killer-cell leukemia (ANKL) is a rare form of large granular lymphocyte leukemia, which is characterized by a systemic proliferation of NK cells. The clinical features of 22 ANKL cases were analyzed. Hepatomegaly (64%), splenomegaly (55%) and lymphadenopathy (41%) were also frequently observed. Leukemic cells were identified as CD1-, CD2+, surface CD3-, CD4-, CD5-, CD7+, CD8+/-, CD10-, CD11b+/-, CD13-, CD16+, CD19-, CD20-, CD25-, CD33(-), CD34-, CD38+, CD56+, CD122+, HLA-DR+ and TCR-. Two of the 16 cases examined for CD57 were positive and three of the seven cases examined for cytoplasmic CD3. Epstein-Barr virus was detected in the tumor cells of 11 of the 13 cases examined. No common cytogenetic abnormalities were identified and 6q anomaly was detected in only one. Three of 13 patients treated with chemotherapy containing anthracycline/anthraquinone attained complete remission, in contrast to none of the eight who were treated with regimens without anthracycline. Although the overall prognosis was poor with a median survival of 58 days, those who attained remission showed better prognosis (P=0.005). These findings suggest that ANKL is an entity of mature cytotoxic NK-cell neoplasms with distinct phenotype and disease presentations. Intensive treatment for ANKL may result in a better prognosis.
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PMID:Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells. 1496 Oct 41

Graft rejection has long been considered the paradigm of renal diseases induced by alloimmunization, particularly alloimmunization directed against HLA antigens. Accumulating evidence indicates that non-HLA immunity also has an important role in clinical transplantation. Targets of alloimmunization include antigens of tubular basement membrane, tubular epithelial cells and endothelial cells. They can be polymorphic allovariants (as shown in the rat) or 'hidden' antigens exposed when the graft is damaged. Alloimmunization can also occur when a person genetically deficient in a renal protein (e.g. the alpha5 (IV) collagen chain in X-linked Alport's syndrome or nephrin in Finnish-type nephrotic syndrome) is transplanted to treat end-stage renal failure. The non-mutated protein in the donor kidney is recognized as a foreign antigen, and the resulting alloimmune response can damage the graft. We have demonstrated that alloimmunity can also affect the native kidney. We have characterized a novel fetomaternal disease in which a genetic defect in the MME gene encoding neutral endopeptidase (NEP) in the mother leads to the development of membranous nephropathy in her fetus (maternal anti-NEP antibodies bind to NEP on fetal podocytes). Our findings raise the possibility that mutations or genetic polyporphisms in MME or other genes expressed by the podocyte are involved in alloimmune-mediated development of membranous nephropathy after kidney or bone marrow transplantation.
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PMID:Mechanisms of disease: Alloimmunization in renal diseases. 1693 67

We report a case of a 64-year-old white female patient, who presented with symptomatic anemia (Hgb: 6.8g/dl), thrombocytopenia (platelets: 94,000/mcl) and leukocytosis (WBC: 156,000/mcl). Peripheral blood smear revealed markedly increased white blood cell count with predominance of atypical lymphoid cells of intermediate size, moderately dense chromatin, and prominent large single nucleoli. Bone marrow aspirate smear showed predominance (78%) of atypical lymphoid cells morphologically identical to those seen in the peripheral blood. The bone marrow core biopsy was hypercellular and packed with prominent infiltrate of prolymphocytes. Immunophenotypic analysis revealed a population of monoclonal cells (75% of all -erythroid cells) characterized by CD45+, CD19+, CD20+, CD5+, HLA-DR+, CD10-, CD23+/-, CD38+ and FMC7-. The abnormal cells were restricted to kappa light chain immunoglobulin with low intensity. Cytogenetic study showed an abnormal clone of eight cells with the following karyotype: 45,X,-X,add(8)(p11.2),t(8;14)(q24;q32),add(20)ql3[8]/46,XX[12]. The relative rarity of B-PLL and the heterogeneity of clinical and laboratory parameters make it difficult to define the natural history and prognosis in all cases. The optimal treatment for B-PLL is still unknown and to date there are no reports of chromosomal abnormalities as a prognostic factor. The patient was treated with six cycles of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP). Complete remission was achieved according to the criteria defined by National Cancer Institute Working Group for CLL.
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PMID:Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia. 1699 73

Leukaemic phase of non-Hodgkin lymphoma (NHL) is characterised by penetration of lymphoma cells from the originating tissues (lymph nodes, less commonly the spleen) into the peripheral blood and bone marrow. The diagnosis of leukaemic phase of Mantle zone lymphoma is established on the basis of histological findings of lymph node biopsy and, possibly, the spleen, peripheral blood smear, and characteristic membranous phenotype. A patient, aged 60, is reported with Mantle zone (intermediate lymphoma) in leukaemic phase. Physical examination revealed pallor of the skin, generalized lymphadenopathy, and hepatomegaly. WBC count in the peripheral blood was 22.5 x 109/l, and the smear revealed the presence of pleomorphic lymphoid cells, mainly medium sized, with irregular nucleus or nuclear notches. Immunophenotype studies of mononuclear cells of the peripheral blood showed characteristic membranous phenotype for Mantle zone lymphoma in leukaemic phase: Smlg+ (lambda light chain); HLA-DR+; CD19+; CD22+; CD5+; CD10-; CD25-. Pro-MACE-Cyta-bom protocol was applied resulting in a 13-month-lasting remission. The total survival was 20 months, suggesting poor prognosis of leukaemic phase of Mantle zone lymphoma.
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PMID:[Leukaemic phase of Mantle zone (intermediate) lymphoma--case report--]. 1797 26


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