Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The cytogenetic, clinical, and immunologic findings ina 4-month-old girl with acute lymphoblastic leukemia (ALL) are reported. The malignant lymphoblasts were characterized cytogenetically by the reciprocal translocation t(11;19)(q23;p13); immunologically by an immature pre-B-ALL phenotype. In spite of the high-risk nature of the leukemia, the patient attained complete remission relatively quickly and is still free of disease 3 years after diagnosis. Because the only two previously reported ALL patients with t(11;19) also seem to have responded well to therapy, this cytogenetic abnormality might turn out to be an indicator of favorable prognosis in ALL.
 ...
PMID:Reciprocal translocation (11;19)(q23;p13) in congenital acute lymphoblastic leukemia. 346 43

Between 1972 and 1982, 112 consecutive previously untreated adults (aged 15-69 years, median 26) commenced therapy for acute lymphoblastic leukaemia (ALL) at St Bartholomew's Hospital. The first 63 patients entered into the study received initial treatment which comprised four cycles of adriamycin and vincristine, prednisolone and L-asparaginase with the first cycle (OPAL). In 1978, six cycles were given, with escalating doses of adriamycin and cyclophosphamide from cycle 3 (HEAV'D). Central nervous system (CNS) prophylaxis incorporated intrathecal methotrexate and cytosine arabinoside with cranial irradiation. Maintenance chemotherapy consisted of 6-mercaptopurine, cyclophosphamide and methotrexate for 3 years. Results obtained with the OPAL and HEAV'D regimens were not significantly different. The overall complete remission (CR) rate was 66% (73/111), factors correlating unfavourably with achievement of CR being advanced age (P less than 0.001) and L3 morphology/B-ALL immunophenotype (P less than 0.01). Fifty-three patients have relapsed, the bone marrow being the primary site in 43. Extramedullary relapse alone occurred in 10 (seven CNS, two testicular and one skin). Only three of the 64 patients who had complete CNS prophylaxis subsequently relapsed in the CNS as an isolated site. One patient died in CR, 19 remain in continuous CR between 2.5 and 10.5 years. The median duration of remission of the 73 patients who achieved CR was 18.5 months, factors correlating favourably with duration of CR being low blast cell count at presentation (P less than 0.002) and common ALL immunophenotype (P less than 0.04). Twenty-four patients remain alive, with a median survival of all patients of 18 months. Long-term survival is possible for approximately 20% of adults with ALL treated relatively intensively.
 ...
PMID:Treatment of acute lymphoblastic leukaemia in adults. 346 41

Long term follow-up of 378 children with acute lymphoblastic leukaemia (ALL) treated at a single centre showed that at six years from diagnosis 202 (53%) were alive, of whom 140 (37%) remained in first remission. Only three children had a first relapse after six years. Children who survived six years despite a single extramedullary relapse in the testis or CNS were likely to remain in second remission but patients with previous marrow or with multiple relapses continued at risk for up to ten years from diagnosis. Presenting factors influencing event-free survival were: leucocyte count, age and sex. After allowing for these factors morphological (FAB) subtype and liver enlargement retained their prognostic significance. Immunological type of ALL was not of independent prognostic significance, except for the small number of patients with B-ALL. Most factors lost their significance after 2-4 years. It is concluded that patients alive 6 years from diagnosis without relapse or even with a single extramedullary relapse of ALL, have a high chance of prolonged survival and cure.
 ...
PMID:Long survival in childhood lymphoblastic leukaemia. 347 Dec 66

Flow cytometry with propidium iodide and fluorescein isothiocyanate was used to study 46 cases of adult acute lymphoid leukaemia (ALL) before any form of chemotherapy. Cell proliferation was related to the other clinical and biological characteristics and its prognostic significance was evaluated. The following cell-cycle variables were determined: S, G2 + M, and the Low Protein Content fraction of G1 (LPC fraction). The L3 group, corresponding to B-ALL, had significantly higher proliferation than L1 and L2 (P less than 0.01). The proliferation rate was not significantly higher for T-ALL than for the other phenotypes. Complete remission was successfully induced significantly more often in cases with the LPC fraction under 50% (P less than 0.05). Failure was mainly related to resistance to chemotherapy. Of the four patients who died during aplasia, three had an LPC fraction below 25%. Duration of complete remission and survival were significantly shorter for L3 which is the most proliferative ALL (P less than 0.01). Survival was also found to be longer (P less than 0.05) when G2 + M was between 3.8% and 5.1%. This finding and the negative correlation between S and G2 + M (P less than 0.01) are discussed.
 ...
PMID:Adult acute lymphoblastic leukaemia: is cell proliferation related to other clinical and biological features? 347 88

The expression of the DNA excision repair enzyme uracil-DNA glycosylase was investigated in bone marrow and peripheral samples from seven patients with acute lymphoblastic leukemia (ALL), from 17 patients with acute non-lymphocytic leukemia (ANLL), and from one patient with chronic granulocytic leukemia (CGL) in blast crisis. In addition, uracil-DNA glycosylase activities were determined in nine human leukemia/lymphoma cell lines. There was a clear correlation between the percentage of blast cells and the enzyme activity when mononuclear cell fractions from patient samples were analysed. The following uracil-DNA glycosylase activities were recorded (mean +/- S.D., number of samples): ALL = 45.6 +/- 14.8 U/mg of protein, N = 10; ANLL = 41.1 +/- 13.8 U/mg of protein, N = 22; CGL (blast crisis) = 44.7 U/mg of protein. The uracil-DNA glycosylase activity in nine human leukemia/lymphoma cell lines ranged from 35.2 to 66.0 U/mg of protein, and no striking differences were observed between the T-ALL, B-ALL, null cell ALL or myeloid lines. Similarly, the various biological features, such as the common ALL surface antigen, the terminal deoxynucleotidyl transferase enzyme, the sub-type of leukemia, chromosomal aberrations, or previous chemotherapy, did not apparently affect the expression of uracil-DNA glycosylase. We propose that the integrity of the genetic information is well protected by uracil-DNA glycosylase in different forms of leukemia, including cases with a low proportion of S-phase blasts, as assessed by flow cytometry in the present work. When compared to the activities in benign hematopoietic progenitor cells, studied previously in this laboratory, no big differences between the benign and malignant hematopoiesis were demonstrated. Hence, it is unlikely that selectivity of chemotherapy towards malignant vs benign hematopoietic growth could be based on the enzyme uracil-DNA glycosylase.
 ...
PMID:Uracil-DNA glycosylase activity in human acute leukemia. 347 82

1255 cases of leukemia-lymphoma were tested between 1972 and 1984 by multiple marker analysis. Routine leukemia phenotyping was performed using standard morphological and cytochemical techniques in combination with clinical and histo-pathological information; the main emphasis was put on immunological surface marker analysis using erythrocyte rosette assays, TdT and a large panel of poly- and monoclonal antibody tests. The 1255 cases were divided into these major types and subtypes: 349 cases of ALL and related immature T- and Burkitt-lymphomas (cALL, pre B-ALL, B-ALL and Burkitt-lymphomas, T-ALL and immature, mostly leukemic T-lymphomas, Null-ALL), 454 cases of mature T- and B-cell malignancies (T-CLL, mycosis fungoides, Sezary-syndrome, T-lymphomas, B-CLL, hairy cell leukemia, multiple myeloma, B-lymphomas), 263 cases of acute myeloid leukemias (AML, AMMoL/AMoL), 182 cases of chronic myeloid leukemias (CML in chronic phase, CMoL, CML in blast crisis), 6 cases of erythroleukemia and 1 case of megakaryoblastic leukemia. A simplified classification scheme which has been used in our laboratories is presented. Phenotyping is of diagnostic, prognostic and therapeutic relevance, most evidently for patients with ALL. Routine leukemia phenotyping should be performed with highly standardized techniques and reagents and by combining information from several fields in the multiple marker analysis. New areas of leukemia research might become very useful for the routine procedure of phenotyping.
 ...
PMID:Phenotyping of malignant hematopoietic cells. Analysis of 1200 cases of leukemia-lymphoma. 348 82

Acute lymphocytic leukemia (ALL) is a heterogeneous group of disorders, clinically, immunologically, and pathologically. ALL of a B cell phenotype (B-ALL) is the least common. We have studied ten adult patients with B-ALL, none of whom had a tumor mass. The median age was 56 years (range, 30 to 90). A history of an altered immune state was noted in four cases: a distant history of Hashimoto's thyroiditis in one, pregnancy in one, and acquired immunodeficiency syndrome in two. Two patients presented with CNS involvement, and in two additional patients CNS leukemia developed during the course of disease. By the French-American-British (FAB) classification system, L3 leukemic morphology was present in nine, whereas L2 was present in one. Circulating leukemic blasts varied from less than 500/dL to greater than 15,000/dL. Eight patients were thrombocytopenic, and eight were anemic at presentation. Immunologic marker studies on leukemic blasts revealed monoclonal kappa light chain marking in nine and monoclonal lambda in one. Following chemotherapy, complete remission was achieved in three patients, two of whom experienced relapse within 9 months. The median survival for the group was 3 months, and only one patient experienced long-term, disease-free survival. We conclude that B-ALL in the adult presents with the classic L3 morphologic picture in the majority and is associated with extremely short survival.
 ...
PMID:B cell acute lymphocytic leukemia in adults. Clinical, morphologic, and immunologic findings. 348 55

A 17-year old caucasian male presented with B-cell acute leukemia which proved aggressive and refractory to treatment. Cytogenic investigation showed a single clone with a complex karyotype 49,XY,del(2)(p13),+4,del(4)(p11),-6,+i(6)(p),+7,+8, t(8;14), (q24;q32),del(17)(p11). This includes the Burkitt's translocation and a deletion at the site of the immunoglobulin kappa light chain gene. Clonal evolution included tetraploidy, duplication of the derived chromosomes and, terminally, trisomy 1q. Immunological investigation revealed a monoclonal population of B-cell blasts, expressing the kappa light chain, and with an extremely rare combination of SIg and TdT positivity. Immunoglobulin gene rearrangement confirmed monoclonalility. Tetraploidy of the clone and del(17)(p11) have been previously described only in a cell line or at end stage disease in B-ALL. It is suggested that the chromosomal abnormalities present at diagnosis were directly related to the refractory nature of this leukemia.
 ...
PMID:Multiple chromosome abnormalities in a drug resistant TdT positive B-cell leukemia. 349 93

The phenotypic marker profile of a patient with B-cell acute lymphoblastic leukemia (B-ALL) is described. The blast cells showed typical FAB L3 morphology, had the characteristic t(8;14) chromosomal abnormality, and were monoclonal in the expression of surface immunoglobulins. The clinical course of this patient is consistent with the poor prognosis described for B-ALL cases. Surface marker analysis identified cells positive for surface immunoglobulins, Ia-like antigen, common ALL-antigen, and terminal deoxynucleotidyl transferase. This marker profile shows immunologic characteristics of the two B-cell leukemia subtypes, pre-B-ALL and B-ALL. This "intermediate" immunologic phenotype might either be the result of an uncoupling of the sequential immunologic maturation processes or of an arrest of the cells at an intermediate stage between the two otherwise clearly defined leukemia subtypes, but closer to the typical B-ALL stage. This latter observation is supported by isoenzyme marker analysis, as the cells were negative for the hexosaminidase I isoenzyme, which is positive in pre-B-ALL but negative in B-ALL.
 ...
PMID:A case of TdT-positive B-cell acute lymphoblastic leukemia. 137 16

After morphology and cytochemistry, immunological leukemic cell diagnosis became a generally available method by the development of monoclonal antibodies. Besides an otherwise not possible classification of nonmyeloid leukemias according to T- or B-differentiation status, precision and standardization of leukemia diagnosis is accomplished by immunodiagnostic means in an experimental way, an important point for therapy studies. In children, the common ALL--a precursor B-ALL type--was prognostically more favorable than the T-ALL which differs also in clinical appearance and in prognostic factors. In adults, however, T-ALL had a better prognosis than common ALL in a prospective trial. The heterogeneous Null-ALL which makes up 23% of ALL in adults and 5% in children, was the prognostically most unfavorable form besides the rare B-ALL (1-2%), the immunophenotype being an independent prognostic variable. Immunodiagnostic means are therefore of utmost clinical importance compared to other methods such as cytogenetic and electron microscopy that are not performable in a corresponding extent.
 ...
PMID:[Leukemia cell analysis in the diagnosis of ALL/AUL: clinical value of currently available methods]. 352 Apr 22


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>