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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the clinical and laboratory findings of 78 adult patients with T-prolymphocytic leukemia (T-PLL) studied over the last 12 years. The main disease features were splenomegaly (73%), lymphadenopathy (53%), hepatomegaly (40%), skin lesions (27%), and a high leukocyte count (greater than 100 x 10(9)/L in 75%) with nucleolated prolymphocytes. A variant form with small, less typical cells was recognized in 19%. Membrane markers defined a postthymic phenotype TdT-, CD2+, CD3+, CD5+, CD7+; in 65%, the cells were CD4+ CD8-, in 21%, they coexpressed CD4 and CD8, and, in 13%, they were CD4- CD8+. Serology for human T-cell leukemia/lymphoma virus Type-I (HTLV-I) was negative in the 27 cases investigated. Cytogenetic analysis in 30 cases showed a consistent abnormality of chromosome 14, usually inv (14), with breakpoints at q11 and q32 in 76% of cases. Trisomy 8, including iso8q, was shown in 53%; t (11;14)(q13;q32) was documented in one case; and one had a normal karyotype. The clinical course was progressive with a median survival of 7.5 months. Thirty-one patients were treated with 2' deoxycoformycin and 15 responded (3 complete remissions and 12 partial remissions); the response rate (48%) increased to 58% in patients with a CD4+ CD8- phenotype. The median survival of responders was 16 months and of nonresponders 10 months; other treatments were less effective. T-PLL is a distinct clinico-pathologic entity with aggressive course and characteristic chromosome abnormalities. A subgroup of patients may benefit from deoxycoformycin.
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PMID:Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. 174 86

Evidence to support the existence of eosinophilic leukemia (EL) as an autonomous eosinophilic proliferation analogous to other myeloproliferative disorders has been somewhat confusing. Partially obscuring the existence of EL as a distinct entity is the proposal that EL merely represents a clinically aggressive form of hypereosinophilic syndrome. This report details the clinical and pathologic findings in a case of EL. The presence of trisomy 8 and trisomy 21; morphologic, cytochemical, and ultrastructural findings of granular abnormalities and nuclear/cytoplasmic dysynchrony; and a clinical course similar to that of other myeloproliferative disorders support the existence of EL as a rare but distinct entity within the spectrum of myeloproliferative diseases.
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PMID:Eosinophilic leukemia: a myeloproliferative disorder distinct from the hypereosinophilic syndrome. 179 68

Surface phenotyping by flow cytometry and cytochemical study were used to identify 15 adult patients with acute leukemia displaying ambiguous phenotypes. Differences were found in the blast cell karyotype and immunoglobulin gene rearrangements of terminal deoxynucleotidyl transferase (TdT)-positive acute myelogenous leukemia (AML) and biphenotypic leukemia expressing B lymphoid and myeloid markers. The karyotypic abnormalities, t(9;22) and t(4;11), were noticed in acute biphenotypic leukemia, and were consistently associated with rearrangement at the immunoglobulin locus. Furthermore, coexpression of CD19/CD20 and either myeloperoxidase or myeloid surface markers were predictive of finding the t(9;22) or t(4;11) karyotype. Patients with TdT-positive AML, on the other hand, were less likely to show rearrangement at the immunoglobulin locus, and did not have the t(9;22) or t(4;11). Instead, a variety of nonrandom karyotypic abnormalities were seen, including trisomy 13. Unlike common AML, the majority of TdT-positive cases demonstrated an abnormal karyotype with duplications and/or deletions present in all cases. In no instance was trisomy 8, t(8;21), t(15;17), or any other isolated translocation identified. The authors therefore suggest that immunophenotyping, when combined with cytochemical analysis of TdT and myeloperoxidase or Sudan black B, may aid in the characterization of subgroups of atypical acute leukemia, such that alternate approaches to therapy can be evaluated.
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PMID:Surface marker analysis and karyotype distinguish acute biphenotypic leukemia from acute myelogenous leukemia expressing terminal deoxynucleotidyl transferase. 191 54

Cytogenetic study in 53 children (aged less than 15 years) with acute non-lymphocytic leukemia (ANLL) were studied. The cytogenetic findings were compared with those of ANLL patients (136 aged less than 19 years and 747 aged over 20 years) in the Fourth International Workshop on Chromosomes in Leukemia (IV IWCL) and also with those of childhood acute lymphoblastic leukemia (ALL) cases (previously reported as our 124 ALL case). Of the ANLL patients, 77.4% had acquired chromosomal clonal abnormalities. As abnormalities, t(15;17), all cases which were seen in M3 or M3V cases, t(8;21), which was seen in M1 or M2, and rearrangements of 11q23, which were seen in M5, were more frequently seen than was reported at the IV IWCL (20.8%, 17.0% and 7.5% vs 6.3%, 6.3% and 3.2% respectively). 5q-, monosomy 7, t(6;9) and t(9;22), which have been noted previously in this disease, were not seen. Besides structural abnormalities, some cytogenetic differences in numerical abnormality between ALL and ANLL were observed as follows: 1) Hyperdiploidy of greater than 51 chromosomes noted in ALL was not found in ANLL. 2) Isolated trisomy 8 was frequently found in ANLL, but not in ALL. 3) Loss of a sex chromosome was frequently found in ANLL, but not in ALL. Our study revealed a different frequency of non-random chromosome abnormality in children with ANLL as compared with that of adults, and clarified the differences in numerical abnormalities, as well as structural abnormalities, between ALL and ANLL.
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PMID:[Cytogenetic studies on 53 childhood acute nonlymphocytic leukemia]. 192 Aug 39

A 62-year-old male with a history of vesical carcinoma treated with pelvic radiotherapy and cystectomy developed intermittent fevers associated with oral ulcers and neutropenia. Serial blood counts revealed cyclic haematopoiesis, with periodic neutropenia, lymphocytopenia, monocytopenia and thrombocytopenia. Bone marrow examination revealed intermittent hypoplasia without myelodysplasia or leukaemia. Marrow karyotype revealed a clonal chromosomal abnormality which included trisomy 8 and absence of the Y chromosome. We also provide evidence of spontaneous differentiation of the clonal marrow cells to mature leucocytes.
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PMID:Acquired cyclic haematopoiesis associated with a radiation-induced chromosomal abnormality with clonal, morphologically normal circulating leucocytes. 195 Mar 69

Chromosome studies were done in 104 patients with various stages of polycythemia vera (PV): 10 had leukemia-myelodysplastic syndrome, 28 had post-PV with myeloid metaplasia (PPVMM), 12 had PV with myelofibrosis, and 54 had PV. Chromosome studies were successful in 86 patients, 37 (43%) of whom had a chromosome abnormality. At diagnosis, 4 of 28 patients (14%) had an abnormal clone; the incidence was 78% in PPVMM and 100% in leukemia-myelodysplastic syndrome. Among the 63 patients with successful chromosome studies during the first 10 years of disease, 27% had an abnormal clone. In contrast, of the 23 patients who had the disease for more than 10 years, 87% had an abnormal clone. Chromosome abnormalities were found in 11 of the 60 patients who either were untreated or underwent only phlebotomy and in 26 of the 44 patients who were treated with myelosuppressive agents. Trisomy 8, +9, and 20q- were found in some patients early during the course of their disease and also among untreated patients. These chromosome abnormalities seem to be related to the natural course of PV rather than to therapy. Patients with a chromosomally abnormal clone at the time of diagnosis of PV had a poorer survival than did those with only normal metaphases. Cytogenetic results did not predict evolution of the disease, but they did provide clues to hematologic phenotype, duration of the disease, and consequences of myelosuppressive therapy.
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PMID:Chromosome studies in 104 patients with polycythemia vera. 200 87

During the 15 year period 1975-1989, 74 cases of chronic myeloproliferative disorder (CMPD) were cytogenetically analyzed in our department. Thirty patients had polycythemia vera (PV), 23 had idiopathic myelofibrosis (MFS), 15 had idiopathic thrombocythemia (IT), and six had unclassifiable CMPD (UCMPD). The overall frequency of clonal chromosome aberrations was 36% (50% in PV, 30% in MFS, 27% in IT, and 17% in UCMPD). The frequency was markedly higher (53%) in the subset of patients who had received myelosuppressive therapy and/or had developed acute leukemia prior to the initial cytogenetic analysis. The pattern of the chromosome rearrangements in our series is in agreement with the karyotypic findings in the 411 previously reported cases of CMPD. Trisomy 8 and 9 and del(20q) dominate in PV. The picture in MFS is more heterogenous with several aberrations, dup(1q), -5, del(5q), -7, del(7q), +8, +9, del(13q), del(20q), and +21, found equally frequently. No pathognomonic chromosome aberration has been detected in IT, but t(9;22) occurs more often than other changes. Thus, although a non-random cytogenetic pattern is discernible in CMPD, there is considerable overlap both with other myeloid malignancies and among the different CMPD subtypes.
Leukemia 1991 Mar
PMID:Karyotypic patterns in chronic myeloproliferative disorders: report on 74 cases and review of the literature. 201 80

A 19-year-old man with a documented 2-year history of Crohn's disease abruptly developed leukopenia and thrombocytopenia. A diagnosis of acute promyelocytic leukemia was established by bone marrow cytology. Chromosomal analysis of bone marrow aspirate revealed aberrations of no. 8 trisomy and translocation between no. 15 and no. 17 [46,XY,t(15q+,17q-)/47,XY,+8,t(15q+,17q-)]. Nine cases of Crohn's disease complicated by leukemia have been reported, including the present one; once again, a relationship between Crohn's disease and leukemia is suggested.
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PMID:Acute promyelocytic leukemia in Crohn's disease. Case report and review of the literature. 206 49

Analysis of the chromosomal changes in various neoplasia is being increasingly carried out not only to evaluate its relationship with the prognosis and biological behaviour of the tumour but also for diagnostic purposes in some cases. Leukaemias are one such group of haematological malignancies which have been most extensively studied in this regard. Karyotypic analysis with Giemsa banding technique was carried out in 35 consecutive cases of Acute Lymphoblastic Leukaemia. Eighteen cases were in children (less than 15 years age) and 17 cases were in adults. M.F. ratio was 1.8:1 FAB classification of these cases showed 31 cases of L1 type and 4 cases of L2 type including one case of T-ALL. Fifteen cases (43%) had no karyotypic abnormality, 6 cases (17%) showed pseudodiploidy, one case each having (-20 + 21), t (9:22), (+ 2-6), (-6 + 8), while two cases had 6 q-.13 cases (37%) showed hyperdiploidy with 6 cases showing trisomy 8 alone, one case (+ 8 + 21), one case trisomy 18, one case + 15(r), one case trisomy 21 plus t (9:22) and one case with trisomy 21 only. Two cases showed more complex abnormalities i.e. + 2 + 8, t (13:22) and -11 + MR + Min + Min. There was one (3%) case of hypodiploidy showing monosomy 6. The above findings are in agreement with studies carried out in other countries except t (13:22) which is rather a scarcely reported abnormality.
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PMID:Chromosomal pattern in acute lymphoblastic leukaemia. 210 33

A case of acute lymphoblastic leukemia (ALL) cytogenetically characterized by trisomy 8 as a sole aberration is described. The patient, one of 116 adults with ALL investigated cytogenetically, was a 36-year-old male with leukocyte count 12.3 x 10(9)/liter with 90% blasts of FAB type L1 and common ALL immunological phenotype. Remission was achieved with the current U.K. treatment trial. The patient recovered from an autologous bone marrow transplant (BMT) in first remission but relapsed 15 months later. BMT, in second remission, from an unrelated donor, was rejected. Autologous reinfusion failed and he died 26 months after diagnosis. Molecular investigation of immunoglobulin gene rearrangement identified the same B cell clone at diagnosis and in relapse. The clinical and cytogenetic findings of six published cases of ALL with trisomy 8 have been reviewed with updates supplied by the authors. These reveal an heterogeneous group of patients ranging in age from 9 months to 39 years with no apparent association with a particular immunophenotype. Four patients were alive after 10-108 months follow-up. Two patients died, in relapse, 7 and 17 months after diagnosis. Thus trisomy 8 occurs in ALL with an incidence of 1-2%. The prognostic significance of this remains to be determined.
Leukemia 1990 Oct
PMID:Trisomy 8 in acute lymphoblastic leukemia (ALL): a case report and update of the literature. 221 75


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