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)

Interferon(rIFN)-alpha, a successful therapeutic agent in the control of thrombocytosis, has been shown to suppress human megakaryopoiesis. We investigated bone marrow biopsies from 12 patients with thrombocytosis due to chronic myeloproliferative disorders. Prior to treatment as well as during rIFN-alpha-2c therapy, several morphometric parameters of megakaryopoiesis were evaluated. Megakaryocyte density decreased significantly in all patients, megakaryocyte size decreased in polycythaemia vera, agnogenic myeloid metaplasia, and essential thrombocythaemia, but increased in chronic myeloid leukaemia. The various changes observed during therapy indicate an inhibitory effect of rIFN-alpha-2c on megakaryopoiesis and suggest a selective influence on megakaryocytes at various stages of maturation. Increased numbers of pyknotic (bare) nuclei may reflect a shortening of megakaryocyte life-span. No remarkable changes were found in the fibre content of the bone marrow.
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PMID:Interferon-alpha-induced morphological changes of megakaryocytes: a histomorphometrical study on bone marrow biopsies in chronic myeloproliferative disorders with excessive thrombocytosis. 231 Jun 90

Our survey on the histology of the bone marrow in chronic myeloproliferative disorders (CMPD) basically gives a description of the relevant lesions of the different subtypes (i.e. chronic myeloid leukemia, primary osteomyelofibrosis, polycythemia vera rubra, and primary thrombocythemia) in correlation with important clinical findings, such as the myelofibrosis/sclerosis syndrome, blast crisis, and prognosis. In a schematic presentation, we assigned the main features of hematopoiesis, interstitial space, and bone tissue to the various subtypes of CMPD, taking into consideration, as well, the evolution of histomorphological lesions in the course of the disease process, which could be determined by means of sequential biopsies. Particularly in the early hyperplastic stages of primary osteomyelofibrosis and in primary (essential) thrombocythemia, we observed a considerable elevation of the platelet count, possibly leading to thrombosis and hemorrhage.
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PMID:[Histomorphologic findings of the bone marrow in chronic myeloproliferative diseases]. 268 47

The content of platelet adenine nucleotide in chronic myeloproliferative disorders (CMPD) and multiple myeloma (MM) was measured by a luciferin-luciferase method by Holmsen and Weiss. The release of ATP and ADP from platelet during aggregation induced by collagen and epinephrine were analyzed. The total 42 investigated cases consisted of 11 cases of polycythemia vera (PV), 7 cases of essential thrombocythemia (ET), 7 cases of chronic myeloid leukemia (CML), 9 cases of blastic crisis of CML (BC-CML), and 8 cases of multiple myeloma (MM). The healthy control was 19 cases. In CMPD and MM, the amount of ATP was normal in spite of decrease of ADP; therefore, the ratio of ATP/ADP increased. On the other hand, the ATP significantly increased in BC-CML. MM revealed a remarkable increase of ATP release due to the aggregation by collagen and epinephrine. The maximal rate of aggregation of collagen and epinephrine using Lumi-aggregometer indicated a positive relationship with the ATP release by the Holmsen and Weiss' method. The platelet volume in CMPD increased showing correlation with ATP content and not with ADP. In conclusion, CMPD and MM are regarded as acquired qualitative disorders of platelets or secondary storage pool diseases from the view points of the abnormalities in ATP, ADP contents and their release. However, BC-CML and MM revealed some different change from that of CMPD.
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PMID:[ATP and ADP of platelets in chronic myeloproliferative disorders and multiple myeloma]. 271 95

In 165 patients with chronic myeloproliferative disorders (CMPD) a morphometric and histochemical study was performed on trephine biopsies of the bone marrow to elucidate osseous remodeling by assessment of trabecular bone area (planimetry) and number of osteoclasts. Osteoclastic elements were identified by the tartrate-resistant acid phosphatase method. In addition to control specimens (n = 20) subtypes of CMPD included chronic myeloid leukemia (CML, n = 65), primary (essential) thrombocythemia (PTH, n = 25), polycythemia vera rubra (P. vera, n = 25) and agnogenic myeloid metaplasia (AMM, n = 50). AMM was discriminated into a so-called early hyperplastic stage without gross myelofibrosis (n = 19) and an overt or advanced stage showing fibro-osteosclerotic changes (n = 31). Total area of trabecular bone and counts for osteoclasts (uni- and multi-nucleated cells as well as a-nuclear cytoplasmic fragments) were not significantly increased in CML, PTH, P. vera and in the initial hypercellular stages of AMM. In contrast to these results, in advanced stages of AMM there was a significant increase in total bone area associated with a high count for all osteoclastic elements and apparently also an increased number of osteoblasts. It is speculated that the marked increase in osteoclastic-osteoblastic elements in late stages of AMM possibly reflects an imbalance of calcitriol (1.25-dihydroxyvitamin D 3) on skeletal homeostasis. This abnormal osseous remodeling may be mediated by the atypical megakaryocytic proliferation in this disorder, which is always a conspicuous feature of bone marrow biopsies.
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PMID:Osteoclasts and bone remodeling in chronic myeloproliferative disorders. A histochemical and morphometric study on trephine biopsies in 165 patients. 278 Apr 31

The incidence of monoclonal gammopathy in 61 patients with chronic myeloproliferative disorders (CMPD) was studied. The distribution of patients among the CMPD subgroups was: chronic myelocytic leukemia, 24 patients; myelofibrosis, 11; polycythemia vera, 15; essential thrombocythemia, 7; unclassified MPD, 4 patients. Monoclonal gammopathy was found in 5 patients (8.2%). Two of these patients (1 IgA/k and 1 IgM/k) had myelofibrosis and 3 (2 IgG/k and 1 IgG/lambda) polycythemia vera. The presence of monoclonal gammopathy indicates an involvement of the lymphoplasmatic system in CMPD.
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PMID:Monoclonal gammopathy in chronic myeloproliferative disorders. 291 5

Acute myelocytic leukemias (AML) are characterized by a remarkably high incidence (approximately 30%) of point mutations affecting codons 12, 13, or 61 within ras genes. A predominant involvement of N-ras sequences has been established. Neither Philadelphia chromosome-positive chronic myelocytic leukemia nor other chronic myeloproliferative disorders show a similar frequency. However, a proportion of myelodysplastic syndromes, namely, the chronic myelomonocytic subtype (CMML) also show this molecular feature. The following is a brief discussion of the possible biologic and clinical implications of these observations.
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PMID:Mutations in ras genes in myelocytic leukemias and myelodysplastic syndromes. 306 84

A histomorphometric analysis was performed on trephine biopsies of the bone marrow in 55 patients with chronic myeloproliferative disorders (CMPDs) and marked thrombocytosis (platelet count exceeding 600 x 10(9)/l). This study aimed at discriminating primary (essential) thrombocythaemia (PTH) from the various other subtypes of CMPDs presenting with thrombocytosis. Following the diagnostic requirements postulated by the Polycythemia-vera-Study-Group for PTH and polycythaemia vera rubra (P.vera) and the generally accepted criteria for the establishment of chronic myeloid leukaemia (CML) and agnogenic myeloid metaplasia (AMM), our cohort of 55 patients was divided into the following subgroups: CML (16 cases), P.vera (11 cases), AMM (13 cases) and finally PTH (15 cases). Histomorphometric measurements revealed that PTH was distinguishable from the other subtypes of CMPDs with respect to several histological variables: patients with PTH had a normal amount of neutrophilic granulo- and erythrocytopoiesis as well as a non-increased content of reticulin (argyrophilic) fibers in contrast to the findings in CML, P.vera and of course AMM. Moreover, sizes of megakaryocytes and their nuclei were significantly greater in PTH and internalization of haematopoietic cells (emperipolesis) was more frequently encountered in comparison with the other subtypes of CMPDs. Deviation of the circular perimeter of megakaryocyte shape was most prominently expressed in CML and AMM, and consequently generated an increased number of a-nuclear cytoplasmic fragments. In contrast to this feature aberration of the nuclei from a circular outline occurred in a less pronounced way in CML, but was excessive in P.vera, AMM and PTH. Our morphometric evaluation demonstrates that certain histological features may serve as a valuable aid in discriminating PTH from the other occasionally thrombocythaemic subtypes of CMPDs.
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PMID:Histomorphometry of bone marrow biopsies in chronic myeloproliferative disorders with associated thrombocytosis--features of significance for the diagnosis of primary (essential) thrombocythaemia. 314 Apr 82

The presence of lymphoid nodules in bone marrow biopsy was investigated at diagnosis in 200 patients with chronic myeloproliferative disorders (MPD). Twelve out of 51 patients with idiopathic myelofibrosis (IM) showed such a feature (23.5%), versus two out of 100 with Ph1-positive chronic myeloid leukaemia, two of 32 with polycythaemia vera, and one of 17 with essential thrombocythaemia, the difference between IM and the remaining MPD being statistically significant (P less than 0.0001). When IM patients were compared for their initial characteristics according to the presence or not of bone marrow lymphoid nodules, patients with such a histological finding showed significantly lower values for either WBC counts, number of primitive cells in the blood, and serum lactic dehydrogenase levels. Moreover, it was observed that virtually all patients with lymphoid nodules were in the nonmyelosclerotic phases of IM. Finally, among the 14 of 32 IM patients (44%) investigated for circulating immune complexes who gave a positive test, a significant association between this immunological abnormality and bone marrow lymphoid nodules was found. The above results reinforce the immunological significance of the finding of bone marrow lymphoid nodules in IM and give support to the hypothesis of an immune component in the pathogenesis of the disorder.
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PMID:Bone marrow lymphoid nodules in myeloproliferative disorders: association with the nonmyelosclerotic phases of idiopathic myelofibrosis and immunological significance. 320 25

The antigenic phenotype of myelomonocytic progenitors [colony-forming unit granulocyte-macrophage (CFU-GM)] from 33 patients with chronic myeloproliferative disorders was investigated using four cytotoxic monoclonal antibodies. Monoclonal antibodies S3-13, S8-6, and S16-144 which recognize normal hemopoietic progenitors of different lineages reacted with almost all CFU-GM. R1B-19 monoclonal antibody identified two subpopulations of myelomonocytic progenitors (type 1 and 2 CFU-GM), as reported previously in normal subjects. In 3 of 11 patients with chronic myelogenous leukemia, in 1 of 2 patients with chronic myelomonocytic leukemia, and in 2 of 4 patients with polycythemia vera, a higher proportion of the more immature CFU-GM (type 1) was detected in bone marrow cells. The more differentiated CFU-GM (type 2) is not detectable in normal peripheral blood. By contrast, in 14 of 15 chronic myelogenous leukemia patients, in 1 of 2 chronic myelomonocytic leukemia patients and in 3 of 8 patients with idiopathic myelofibrosis, it was present in high to very high proportions. It is clear from these findings that the antigens present on normal CFU-GM are expressed in chronic myeloproliferative disorders. The proportion and distribution of type 1 and 2 CFU-GM, on the other hand, are very different from those observed in the normal subjects.
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PMID:Antigenic phenotype of myelomonocytic progenitors (CFU-GM) in chronic myeloproliferative disorders. 345 79

Bone marrow biopsies of 850 patients with chronic myeloproliferative disorders were taken at initial diagnosis; and 169 sequential biopsies over periods of one to 188 months. Three micron sections of all biopsies were evaluated semiquantitatively with reference to the proliferating cell lines, anomalies of megakaryocytes, and fibrosis or osteosclerosis. Correlations between initial histological findings, clinical, haematological, and survival data were analysed statistically. The predominant cell lines distinguished the classical entities of polycythaemia vera, primary thrombocythaemia, and chronic myeloid leukaemia and correlated with their different prognoses, while megakaryocytes characterised subgroups that were prone to fibrotic or blastic transformation. Based on the initial histological, clinical, and haematological data analysed a working classification of chronic myeloproliferative disorders was proposed that permits recognition of both typical and atypical cases of chronic myeloproliferative disorders.
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PMID:Working classification of chronic myeloproliferative disorders based on histological, haematological, and clinical findings. 345 24


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