Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The therapeutic efficacy and toxicity of alpha-interferon (alpha-IFN) (Roferon, Hoffmann-La Roche, Inc., Nutley, NJ) were determined in 15 children (age range, 6 to 20 years) with Philadelphia chromosome-positive chronic myelocytic leukemia (Ph+ CML). All patients had received cytoreductive therapy with either hydroxyurea (n = 13) or busulfan (n = 1) or both (n = 1) for 6 weeks to 46 months (median, 7 months) before beginning alpha-IFN therapy at a dose of 5 x 10(6) U/m2/d intramuscularly. This dose was escalated to 10 x 10(6) U/m2/d if leukemia was inadequately controlled. Ten children had a hematologic response, with nine showing a reduction in the percentage of Ph+ marrow cells, including four who had no detectable Ph+ cells in marrow samples collected 48 to 204 weeks after the initiation of therapy. Two of 15 patients remain free of Ph+ cells. Therapy was discontinued before week 104 in ten patients because of the following: (1) early hematologic responses without a decrease in Ph+ cells (three patients); (2) early resistant disease (one patient); (3) blast crisis (one patient); (4) progressive disease (two patients); (5) seizure attributed to high-dose alpha-IFN (one patient); or (6) an inadequate trial of alpha-IFN caused by aseptic necrosis or poor compliance (two patients). The most common side effects were mild and have included fever, malaise, headache, myalgias, and pain at the injection site. Adverse events causing interruption of therapy were seizures, aseptic necrosis, and myelofibrosis. alpha-IFN stabilizes the chronic phase of Ph+ CML in some children, is adequately tolerated when administered at a dose of 2.5 to 5 x 10(6) U/m2/d intramuscularly, and results in a significant decrease in the proportion of Ph+ metaphases in some patients. alpha-IFN in combination with an effective cytoreductive agent or agents appears worthy of further clinical testing in this disease.
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PMID:Response to alpha-interferon in children with Philadelphia chromosome-positive chronic myelocytic leukemia. 183 44

Idiopathic myelofibrosis is reviewed from several aspects. The historical development of knowledge about this disorder is discussed, from early descriptions of extramedullary hematopoiesis associated with numerous etiologies, a debate over pathogenetic mechanisms, followed by newer evidence which placed this disorder with the myeloproliferative disorders. Evidence is presented showing that idiopathic myelofibrosis is an acquired clonal disorder in terms of the hematopoietic abnormalities, but that the marrow fibrosis is a result of non-clonal disordered fibrogenesis. The clinical, laboratory and pathologic features of idiopathic myelofibrosis are discussed. The features which distinguish this disorder from the other myeloproliferative disorders, particularly chronic myelogenous leukemia are emphasized. The natural history is described together with an evaluation of accepted and experimental therapy.
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PMID:Idiopathic myelofibrosis: historical review, diagnosis and management. 191 60

One hundred and seventy bone marrow biopsies from patients with chronic myeloproliferative disorders (CMPDs) were evaluated for the presence of lymphoid nodules (LNs) and were immunostained using a panel of monoclonal antibodies (UCHL1, 4KB5 and L26) recognizing different lymphocyte antigens. LNs were found in 35% of cases of idiopathic thrombocythaemia, 24.6% of myelofibrosis/osteomyelosclerosis, 18.2% of polycythaemia vera 12.1% of chronic myeloid leukaemia and 19.2% of borderline cases. Varying degrees of immunohistochemical positivity for the three antibodies tested were found. LNs were always made up of variable proportions of both T- and B-lymphocytes with a prevalence of T-cells. This latter observation suggests that bone marrow LNs in CMPDs could be an expression of reactivity.
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PMID:Immunohistochemical evaluation of bone marrow lymphoid nodules in chronic myeloproliferative disorders. 194 8

The myeloproliferative disorders comprise a group of related diseases, including polycythemia vera, essential thrombocythemia, chronic myelogenous leukemia, myelofibrosis, and myeloid metaplasia. An increase in circulating platelets is associated with thrombotic phenomena affecting the arterial and venous circulation. In this article, the authors describe a case in which the initial manifestation of myeloproliferative disease was gangrene of the fingers.
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PMID:Gangrene of the fingers secondary to myeloproliferative disease. 206 51

The purpose of the study was assessment of the effectiveness of treatment applied in nine proliferative diseases of the haemopoietic system (PDHS) in the years 1951-1980. The effectiveness was determined comparing the mean survival time in each of these diseases in three 10-year-time periods characterised by essential changes in their treatment. Moreover, other factors were studied which may influence the survival time in these diseases. A continuing increase in the survival time correlated with advances in therapy was observed in acute myeloid leukaemia (AML), acute lymphoblastic leukaemia (ALL), Hodgkin's disease (HD) and multiple myeloma (MM). An indicator of the advances in the treatment of acute leukaemias was also an over fourfold rise in the likelihood of achieving complete remission in the decade 1971-1980 in relation to two preceding decades. On the other hand, no improvement of the effectiveness of treatment was noted in chronic myeloid leukaemia (CML), polycythaemia vera (PV), myelofibrosis (MF) and non-Hodgkin lymphomas (NHL). The length of the survival time was influenced also considerably by patient's age (survival lower in old age), sex (better results in women) and place of residence of the patient (worse results in patients living in rural areas).
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PMID:Clinical-haematological analysis of 3321 cases of proliferative diseases of the haemopoietic system treated in the Institute of Haematology in the years 1951-1980. 207 60

Acute megakaryoblastic leukemia (AMkL) is a newly defined acute leukemia in which the differentiation of proliferating blasts is arrested at the megakaryocytic precursor stage. In order to clarify whether a target cell of leukemic transformation in AMkL is a cell committed to megakaryocytic lineage, or a multipotential stem cell, we examined AMkL patients with regard to: a) the presence of myelodyplastic features in residual erythroid and granulocytic cells, b) coexistence of myeloperoxidase (MPO)-positive blasts with megakaryoblasts, and c) the presence of the same chromosomal abnormality in erythroid and granuloid colony-forming cells as seen in megakaryoblasts. Regarding the former two items, results were compared with those from megakaryoblastic crisis of chronic myelocytic leukemia (CML-MkBC) and transient myeloproliferative disorder in Down syndrome (DS-TMD), which are thought to be multipotential stem cell disorders. Among 18 patients with AMkL, three, all complicating myelofibrosis, had marked myelodysplastic changes of erythroid series and/or granulocytic series. In 4 out of 7 patients with CML-MkBC, 5 out of 8 patients with DS-TMD, and 7 out of 18 patients with AMkL, MPO-positive blasts, even though rare, were observed in addition to PPO-positive blasts. All except one of these patients with AMkL also showed complicating myelofibrosis. In one case of AMkL with myelofibrosis, chromosomal analysis of cultured cells of individual colonies revealed that all the analysable metaphases from both CFU-GM and BFU-E had the same chromosomal abnormality as megakaryoblasts. This study has clarified that a considerable proportion of AMkL cases, particularly those with complicating myelofibrosis or showing acute myelofibrosis, arise against the background of a multipotential stem cell disorder, even if blasts are exclusively megakaryocytic in phenotype.
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PMID:Target cell of leukemic transformation in acute megakaryoblastic leukemia. 216 21

This Diagnostic Seminar intends to announce that CMPDs can be classified from BMB histologically by a rather simple system, which can be applied by interested histopathologists successfully. The rationale of this classification is to stay within the groups of diseases which are outlined by clinical findings including the peripheral blood and bone marrow smears. The concept of traditional classification as given by the WHO and textbooks, however, has to be revised as follows (1) Primary diseases of CMPDs must be distinguished from advanced disorders. Primary diseases are CML, P. vera, Thrombocythemia, CMGM, and unclassifiable CMPD. (2) Idiopathic, primary myelosclerosis of the bone marrow is a reactive feature consecutive to neoplastic transformation of hematopoiesis, i.e. myeloproliferation. (3) Advanced disorders comprise (3.1.) excess of blasts and blast crisis, and (3.2.) early myelosclerosis, myelosclerosis and myelofibrosis, advanced myelofibrosis. Advanced disorders are designated by a composed term classifying them among the groups of primary disease and specifying the advanced stage by a suffix, so that the underlying disease remains coining the term, even in unclassifiable cases in which only CMPDs can be applied. (4) The CML group must be subtyped into CML of common type versus that with increase or predominance of megakaryocytes. By this system of classification, it seems possible to classify and type the spectrum of variations occurring among CMPDs to a satisfying result.
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PMID:Chronic myeloproliferative disorders in bone marrow biopsies. 217 9

Leukocytosis, mild anemia, thrombocytosis, and panhyperplasia in the marrow characterize the early stages of most of the CMPD, whereas extramedullary hematopoiesis (such as in the spleen or liver), peripheral cytopenias (anemia, leukopenia, or thrombocytopenia), and myelofibrosis, with or without osteosclerosis, reflect the changes seen in the later stages. Transitions among the different CMPD and termination in acute leukemia or marrow failure also are common. CML often is characterized by leukocytosis and the presence of the entire spectrum of granulocytes (mature and immature) in the blood and marrow, reduced LAP, hypercellularity with prominent granulocytic hyperplasia in the marrow, Ph chromosome, and bcr-abl gene rearrangement. Typical features of AMM include leukoerythroblastosis, teardrop poikilocytosis, anemia, increased or normal LAP, prominent megakaryocytic hyperplasia in the marrow, dyshematopoiesis, and hyperplastic or fibrotic/sclerotic marrow.
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PMID:Myeloproliferative disorders. Classification and diagnostic features with special emphasis on chronic myelogenous leukemia and agnogenic myeloid metaplasia. 227 76

A morphometric study was performed on trephine biopsies of bone marrow in patients with chronic myeloid leukemia (CML) accompanied by myelofibrosis and in so-called primary (idiopathic) osteomyelofibrosis/-sclerosis (OMF) to evaluate distinctive features of megakaryopoiesis. The periodic acid Schiff reaction (PAS) and a monoclonal antibody against glycoprotein IIIa were employed for the identification of megakaryocytes including precursor cells and Gomori's silver impregnation to determine the density of argyrophilic fibers. All patients with CML revealed a slight to moderate degree of medullary fibrosis and were compared with early hyperplastic stages of OMF showing an identical fiber count. Statistical analysis disclosed that distinctive features existed between these two subgroups. Amongst these variables were sizes of megakaryocytes and corresponding nuclei, frequency of bare nuclei, emperipolesis and numbers of isolated nuclear fragments as well as the circular deviation of cell and nuclear perimeters. Immunomorphometry also included immature elements (pro- and megakaryoblasts) of the megakaryocyte series. Consequently higher cell counts were calculable in both groups combined with smaller sizes and a more rounded aspect of nuclei. However, following immunostaining, significant differences in several megakaryocytic parameters (frequency, size, shape of nuclei) were still demonstrable between CML and OMF cases.
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PMID:Histo- and immunomorphometry of megakaryopoiesis in chronic myeloid leukemia with myelofibrosis and so-called primary (idiopathic) osteo-myelofibrosis/-sclerosis. 227 69

Cytogenetic analyses were performed on 12 adult patients with abnormal megakaryoblastic proliferation which was detected by ultrastructural cytochemical study (platelet peroxidase) and platelet-megakaryocytes-specific monoclonal antibodies (TP-80, Plt1, AN51, and KOR-77). The patients consisted of two patients with myelodysplastic syndromes (MDS), three with acute megakaryoblastic leukemia (AMKL), six with megakaryoblastic transformation in Philadelphia-positive chronic myelogenous leukemia (CML-meg-BC), and one case of chronic myeloproliferative disorder (CMPD). Among them, an inversion of the long arm of chromosome 3 [inv(3)(q21q26)] was found in one AMKL patient with a normal platelet count. Chromosome change at band 3q26 was also found in one MDS patient without thrombocythemia. Furthermore, the long arm of chromosome 13, where rearrangements in myelofibrosis are clustered (13q12----q22) was seen in one MDS patient. Trisomoy of chromosome 19 was found in one AMKL patient and three CML-meg-BC patients. These findings indicate that cytogenetic abnormalities involving 3q26, 13q, and trisomy 19 are associated with hematologic neoplasia with megakaryocytic lineage in adult patients, although these abnormalities were not related to the survival of the patients. During the period of this study, two acute myelogenous leukemia patients (AML-M2 and AML-M5b) with chromosome rearrangements at band 3q21 and thrombocythemia were found, indicating that chromosome abnormality at band 3q21 is related to quantitative platelet dysfunction, whereas that at 3q26 is related to hematologic malignancies with a proliferation of megakaryocytic lineage.
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PMID:Cytogenetic findings in adult acute leukemia and myeloproliferative disorders with an involvement of megakaryocyte lineage. 229 63


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