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 authors report a patient with coexistent double gammopathy, a Philadelphia chromosome-negative, bcr rearrangement-negative myeloproliferative disease resembling chronic myelocytic leukemia and a malignant lymphoma of B-cell origin. The double gammopathy consisted of IgM (kappa) and IgG (kappa). Peripheral blood, spleen, and marrow lymphocytes had primarily an IgG (kappa) isotype, whereas lymph node lymphocytes had predominantly an IgM (kappa) surface isotype. Increased numbers of marrow lymphocytes stained doubly for both IgM (kappa) and IgG (kappa). The results suggest that doubly isotypic as well as single isotypic lymphocytes contributed to the double gammopathy. Organ localization differed for lymphocytes with different antibody isotypes. This cluster of findings has not been described previously.
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PMID:Coexistent double gammopathy, myeloproliferative disorder, and malignant lymphoma. 210 37

During the past 12 years we studied children with unexplained chronic leukocytosis and other findings suggestive of acute or chronic myeloid leukemia (AML; CML). We used cultures in soft agar of peripheral blood for granulocyte-macrophage colony-forming cell (GM-CFC) analysis. Colonies were counted, examined morphologically and cytochemically and the findings in patients were compared with those in normal children and patients with leukemoid reactions. 2 children with confirmed CML and neurofibromatosis (NF) were similarly evaluated. Additional studies in 1 of them and in his mother who had NF, included establishment of fibroblast and blood cultures from affected skin and tumor, and stimulation of normal bone marrow-derived GM-CFC by these fibroblasts and the conditioned medium (CM) from these cultures. Growth of GM-CFC from blood cultures of CML and AML patients was significantly enhanced in comparison with blood cultures from normal donors, or patients with other myeloproliferative disorders or leukemoid reactions. Enhanced GM-CFC growth-supportive activity was obtained from CML-NF skin and tumor culture CM in comparison with CM from normal fibroblasts. These results indicate the diagnostic value of blood culture GM-CFC in juvenile CML, and its usefulness in differentiating between CML and other disorders involving leukocytosis. They suggest a possible connection between NF foci and the enhanced proliferation of blood GM-CFC in CML.
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PMID:[Blood culture for diagnosing juvenile chronic myeloid leukemia; relationship to neurofibromatosis]. 210 34

Interferon alfa has been used in the treatment of myeloproliferative disorders, particularly chronic myeloid leukemia, polycythemia vera, and idiopathic thrombocythemia. The effectiveness of interferon alfa in agnogenic myeloid metaplasia needs additional evaluation, although preliminary evidence suggests that it may be more efficacious when used in the cellular (ie, proliferative) phase than when the marrow is fibrotic or osteosclerotic. Cytogenetic and molecular changes after interferon alfa therapy are apparent in patients with chronic myeloid leukemia, as manifested by change in the Philadelphia chromosome and BCR-ABL gene, respectively. The exact role of interferon in prolonging the life of chronic myeloid leukemia patients, however, remains to be determined in larger studies of longer duration. Interferon treatment seems to be well tolerated, and the frequency of treatment-limiting toxicity is low. Data to date suggest that interferon alfa may be a new and effective drug for the treatment of the myeloproliferative disorders.
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PMID:Interferon in the treatment of myeloproliferative diseases. 211 94

Blood findings in 61 cases of generalized mastocytosis (GM) were evaluated. The cases were divided into two major variants: Systemic mastocytosis (SM; n = 34) with urticaria pigmentosa-like skin lesions, and malignant mastocytosis (MM; n = 27), without skin involvement. The following results were obtained: (1) Significant differences between MM and SM were found in the main haematological parameters (erythrocyte, platelet and leucocyte counts and haemoglobin level); normal values were found in 16 of the SM cases, but never in MM. (2) The main pathological findings were: in SM, anaemia (9/34) and leucocytosis (5/34); and in MM, leucocytosis (19/27), monocytosis (14/27), eosinophilia (12/27), bicytopenia (12/27, mostly anaemia with thrombocytopenia), basophilia (10/27) and isolated anaemia (7/27). (3) The major finding was a significant difference between MM and SM in the incidence of myeloproliferative disorders (MPD), myelodysplasia and mast cell leukaemia (MCL): these disorders occurred in 23 (92%) MM patients, but only in two (6%) SM patients (P less than 0.001). The four instances of MCL and two of myelodysplasia all occurred with MM. Of the 19 cases of MPD, six (SM, 1; MM, 5) were acute variants (acute myeloid and myelomonocytic leukaemias) and 13 (SM, 1; MM, 12) were chronic variants. No case of malignant lymphoma was noted. (4) The blood picture in 10 of 13 chronic MPD cases represented an atypical chronic myeloid leukaemia for which the preliminary descriptive term 'mastocytosis-associated MPD' is proposed. (5) A survey of 103 published cases (SM, 77; MM, 26) yielded similar findings, including a high incidence of MPD and MCL in MM. These findings add further weight to the argument for recognizing SM and MM as two separate entities.
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PMID:Blood findings in generalized mastocytosis: evidence of frequent simultaneous occurrence of myeloproliferative disorders. 201 71

Forty-one patients with myeloid malignancies, majority newly diagnosed and previously untreated, were systematically karyotyped between January 1988 and December 1989 using fluorodeoxyuridine (FdU) synchronisation and Giemsa banding techniques. Eighteen patients had acute myeloid leukaemia (AML), 13 had one form or other of a myeloproliferative disorder and 10 fulfilled the clinical and morphological criteria for the myelodysplastic syndrome (MDS). Ten AML patients had cytogenetic abnormalities. All 6 chronic myeloid leukaemia (CML) patients carried the Philadelphia chromosome with 2 having additional abnormal clones. Only four MDS patients showed involvement of either chromosome 5 or 7.
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PMID:Cytogenetic analysis in myeloid malignancies. 213 Jul 41

Platelet function and the clinical course of the disease were prospectively investigated in 29 patients with myeloproliferative disorders. Serial determinations (median: 5 investigations per patient within 17 months) of platelet aggregation, plasma and intraplatelet concentrations of beta-thromboglobulin (beta TG) and platelet factor 4 (PF4), and of fibrinopeptide A (FPA) plasma levels were carried out. In the chronic phase of polycythaemia vera, patients with thrombohaemorrhagic complications during the study period had higher platelet count, more severe platelet aggregation defects, and increased plasma levels of beta TG and FPA compared to patients without complications. However, thrombohaemorrhagic complications were not predicted by changes in these parameters in the individual patient during the chronic disease phase. When patients with chronic myelogenous leukaemia entered blast crisis, bleeding complications were related to thrombocytopenia, impaired platelet function and low intraplatelet concentrations of beta TG and PF4. Cytoreduction by chemotherapy in the chronic phase of CML did not alter beta TG and PF4 plasma levels, whereas treatment of polycythaemia rubra vera by venesection favourably influenced platelet alpha-granule secretion and increased intraplatelet concentrations of beta TG and PF4.
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PMID:A prospective study of haemostatic parameters in relation to the clinical course of myeloproliferative disorders. 214 44

Trisomy 21 as an acquired clonal chromosome change has been described in 642 of the 10,625 human neoplasms with chromosome aberrations known from the cytogenetic literature. A total of 590 of the 642 cases (92%) are hematologic disorders and malignant lymphomas. The incidence of trisomy 21 is similar (4.1%-6.7%) in acute myeloid leukemia (AML), chronic myeloid leukemia, myeloproliferative disorders, myelodysplastic syndromes, chronic lymphoproliferative disorders, and malignant lymphomas; it is substantially higher (14.8%) in acute lymphocytic leukemia (ALL). In most cases, the extra chromosome 21 is present together with other numerical and/or structural changes. Acquired trisomy 21 is the only karyotypic abnormality in only 0.4%. Trisomy 21 has never been reported as the sole anomaly in a solid tumor. The cytogenetic literature contains information on 62 patients with constitutional trisomy 21 and a malignant disorder in which the tumor cells have been analyzed by banding techniques. Thirty-four of the 62 patients had AML, 16 had ALL, and 2 had acute undifferentiated leukemia. The 52 leukemic Down syndrome (DS) cases account for 1.4% of the total acute leukemias, an overrepresentation that parallels the generally increased risk of leukemia development in DS. Sixty-three percent of the ALL patients and 79% of those with AML had additional changes superimposed on constitutional trisomy 21. These included several of the characteristic primary leukemia-associated aberrations: 5q-, 7q-, +8, and t(8;21) in AML, and t(1;19), t(4;11), 6q-, and 14q + in ALL. Thus, it seems that the pattern of acquired karyotypic changes is similar in patients with DS and in individuals with a normal constitutional karyotype.
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PMID:Trisomy 21 in neoplastic cells. 214 59

An enzyme-linked immunosorbent assay using specific monoclonal antibodies was used to measure circulating transferrin receptor (TR) in 87 patients with various hematologic malignancies. The mean serum TR was significantly elevated in patients with myeloproliferative disorders (15.47 +/- 12.54 micrograms/ml), whereas there were no differences in chronic granulocytic leukemia (7.89 +/- 3.56 micrograms/ml), myelodysplastic disorders (9.25 +/- 4.73 micrograms/ml), and acute nonlymphocytic leukemia (3.85 +/- 3.50 micrograms/ml) as compared to normal (5.63 +/- 1.42 micrograms/ml). Among patients with lymphoproliferative disorders, the mean level was normal in lymphoma (5.73 +/- 2.59 micrograms/ml), multiple myeloma (5.47 +/- 1.31 micrograms/ml), and hairy cell leukemia (7.04 +/- 3.69 micrograms/ml). The serum TR was significantly elevated in chronic lymphocytic leukemia (CLL; 14.17 +/- 12.29 micrograms/ml), and the serum levels reflected the clinical stage of the disease. These findings suggest that serum TR measurement may provide a useful laboratory index of disease activity in certain disorders such as CLL, whereas it most likely reflects the intensity of erythropoiesis in the remaining hematological disorders that were evaluated in this study.
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PMID:Serum transferrin receptor measurements in hematologic malignancies. 216 85

Chronic myelogenous leukemia (CML) is a myeloproliferative disorder triggered by a chromosomally induced mutation in a pluripotent stem cell. Its progressive clinical course consists of a chronic or benign phase, which terminates in blast crisis. Historically, treatment has been limited: conventional chemotherapy yields a median survival of approximately 36 to 42 months, and bone marrow transplantation, while achieving success in more than half of patients treated, is limited to younger patients with HLA-matched siblings. Treatment with interferon alfa in CML was initiated in 1981. The first studies were performed using partially pure interferon alfa; these studies were followed by treatment regimens employing interferon alfa-2a.
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PMID:Update on therapeutic options for chronic myelogenous leukemia. 219 31

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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