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)

Bcr-abl mRNA expression was studied in patients with chronic myeloproliferative disorders (CMPD) by the reverse transcriptase-polymerase chain reaction (RT-PCR) method. A bcr-abl transcript was not found in any patient with polycythemia vera, essential thrombocythemia or primary myelofibrosis, suggesting that the bcr-abl rearranged clone is not present in CMPD other than chronic myelogenous leukemia (CML). In CML clinical and laboratory data were compared from three bcr-abl types: the bcr exon 2-abl exon 2 (B2-A2) type, bcr exon 3-abl exon 2 (B3-A2) type and the co-expression type. Age at diagnosis tended to be younger (p = 0.08) in the co-expression type, and the platelet count tended to be lower (p = 0.11) in the B2-A2 type. However, there was no difference in other data, including the duration of the chronic phase and the phenotype of blasts at blast crisis.
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PMID:Bcr-abl mRNA expression in patients with chronic myeloproliferative disorders--absence of bcr-abl fused clone except chronic myelocytic leukemia. 825

The distribution and the development of fibrosis were evaluated from bone marrow biopsies of patients with chronic myeloproliferative disorders (CMPD), regarding two groups of patients: (1) 564 with follow-up biopsies over a period of up to twelve years observation time, and (2) 1.787 diagnostic bone marrow biopsies from CMPD patients. Fibrosis was divided into three grades of fiber increase: early myelosclerosis, myelofibrosis, and advanced myelofibrosis. The first group of sequential BMB showed a significant progress to myelofibrosis in so-called "Chronic Megakaryocytic-Granulocytic Myelosis"--CMGM-, which corresponds to Agnogenic Myeloid Metaplasia-AMM-in 72.4% (21/29 patients), as well as in CML with megakaryocytic increase-CML.MI-in 39.2% (20/51). In the second group of diagnostic biopsies, only 30% of CMGM cases showed no fibrosis. In P. vera, 16.2% (18/111) developed myelofibrosis up to twelve years later. This figure was 4.3% (2/46) in Primary Thrombocythemia. Increase of megakaryocytes in CML indicates a high risk for developing fibrosis, combined with reduced life expectancy.
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PMID:Myelofibrosis in chronic myeloproliferative disorders. Incidence among subtypes according to the Hannover Classification. 832 41

Diagnosis of chronic myeloproliferative disorders (CMPD) can encounter difficulties due to overlaps and possible transitions between the different entities and their similarity to reactive myeloproliferations. In this study DNA analysis has been applied to improve differentiation of CMPD. All subtypes of CMPD analyzed, including chronic myeloid leukemia (CML), agnogenic myeloid metaplasia (AMM), polycythemia vera (PV), and essential thrombocythemia (ET), had in common that granulocytes and bone marrow cells were clonal in origin as shown by X-chromosome-linked DNA polymorphism in conjunction with methylation patterns. Reactive myeloproliferations, by contrast, revealed a polyclonal inactivation pattern. Clonality could not distinguish CMPD from cases of myelodysplastic syndrome (MDS) since the latter also exhibited clonal hematopoiesis. AMM belongs to the group of myeloproliferative syndromes. Up to now the cellular phase at onset of the disease (megakaryocytic myelosis) has not been analyzed for clonality of the hematopoietic cells. Granulocytes as well as bone marrow cells from the cellular phase and advanced stages of the disease revealed a monoclonal inactivation pattern of X-chromosomal genes. These results show that the cellular phase already represents a monoclonal, and hence probably a neoplastic, proliferation of a pluripotent stem cell. The monoclonality of granulocytes could also be demonstrated in patients with splenomegaly and strongly argues against a compensatory proliferation of regular hematopoiesis in this organ. Because of their clonal origin, peripheral granulocytes were used in all cases (n = 244) for the detection of bcr-gene rearrangement. Despite possible morphological overlaps between different types of CMPD, bcr-gene rearrangement proved to be specific for CML and could be applied to differentiate CML from other CMPD in cases of uncertain morphological diagnosis. It is concluded that CMPD represent clonal hemopoietic disorders that probably have specific underlying genetic defects. Thus, DNA analysis can substantially aid in the differential diagnosis of CMPD.
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PMID:[Histopathology and molecular pathology of chronic myeloproliferative disorders]. 837 86

We examined the expression of c-Mpl (MPL) and c-Mpl ligand (ML) gene in hematopoietic cells in individuals with and without myeloproliferative disorders (MPD) and leukemic cell lines by RT-PCR. The MPL gene transcripts were detected in normal CD34+ cells, platelets, megakaryocytes and monocytes, while the ML gene was expressed in CD34+ cells, megakaryocytes, T cells, monocytes and bone marrow fibroblasts, as well as liver tissue. The ML gene product produced in the bone marrow microenvironment might, in part, be involved in hematopoiesis. The MPL gene expression was detected in platelets and peripheral blood mononuclear cells from the majority of patients with MPD including chronic myelocytic leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). In contrast, the ML gene expression was found in the majority of ET and CML patients, but not in PV or PMF patients. These findings suggest that even in MPD the megakaryocytopoiesis depends on the MPL signal transduction system, and that in ET and CML, the ML production by mononuclear cells in the bone marrow microenvironment might play a part in the higher megakaryocytopoiesis observed in these diseases. Both the MPL and ML gene expression were detected in all the leukemic cell lines tested, suggesting that this cytokine/receptor system is involved in cell growth through autocrine and paracrine systems.
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PMID:Expression of c-Mpl and c-Mpl ligand gene in hematopoietic cells of individuals with and without myeloproliferative disorders and leukemia cell lines. 858 67

The urinary concentration of calmodulin and basic fibroblast growth factor (bFGF) was determined in a total of 53 patients with various chronic myeloproliferative disorders (CMPD), including 22 patients with idiopathic myelofibrosis (IMF). Calmodulin excretion was significantly elevated in IMF (0.29 +/- 0.04 microgram/mmol creatinine) (P < 0.001), when compared to polycythaemia vera (PV) (0.14 +/- 0.02), essential thrombocythaemia (ET) (0.13 +/- 0.04), chronic myeloid leukaemia (CML) (0.16 +/- 0.02), unclassified myeloproliferative disorders (UMPD) (0.11 +/- 0.02) and age-matched controls (0.1 +/- 0.02) (P < 0.001). In contrast, bFGF was slightly elevated in all CMPD conditions when compared to age-matched controls. A neutralizing antibody to calmodulin was demonstrated to significantly influence the in vitro proliferation of normal human fibroblasts, an effect dependent on both cell density and the presence of fetal calf serum (FCS). Essentially, the antibody reduced FCS-induced proliferation of low-density fibroblasts but had little or no inhibitory effect on high-density fibroblasts in the absence of FCS. In addition, extracellular calmodulin was shown not to interact with known fibroblast mitogens, namely, IFG-1, EGF, bDGF and PDGF. We conclude that extracellular calmodulin should be considered, in addition to PDGF, TFG-beta and EGF, as a potential mitogen involved in the stromal reaction of idiopathic myelofibrosis.
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PMID:Investigation of calmodulin and basic fibroblast growth factor (bFGF) in idiopathic myelofibrosis: evidence for a role of extracellular calmodulin in fibroblast proliferation. 870 17

Myeloproliferative disorders are clonal disorders of the hematopoietic stem cell and comprise a spectrum of more or less well-defined clinical entities: polycythaemia vera, chronic myeloid leukemia, essential thrombocythaemia, and agnogenic myeloid metaplasia. Myelofibrosis, which contributes substantially to the impaired hematopoiesis, is commonly observed in myeloproliferative disorders but it represents the criterion of agnogenic myeloid metaplasia also termed idiopathic myelofibrosis. Although progress has been made in the elucidation of the pathogenesis of myelofibrosis, it still remains unclear. The aim of this review is to address the new insights that outline the potential role of TGF-beta in the promotion of myelofibrosis, through its release from megakaryocytes/platelets, particularly in agnogenic myeloid metaplasia.
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PMID:TGF-beta and megakaryocytes in the pathogenesis of myelofibrosis in myeloproliferative disorders. 875 Jun 21

The bcr gene rearrangement resulting from the Philadelphia translocation is diagnostic of chronic myelogenous leukemia (CML) and is considered the hallmark of this myeloproliferative disorder (MPD) at the molecular level. The other MPDs, essential thrombocythemia (ET), polycythemia vera (PV), agnogenic myeloid metaplasia (AMM), and unclassified MPD, share morphologic features with CML, making the diagnosis difficult in cases with considerable morphologic overlap. In such cases, molecular analysis becomes essential for accurate diagnosis. We report results of bcr analysis by Southern hybridization in 37 patients with MPDs other than CML: ET (20 cases), PV (seven cases). unclassified MPD (nine cases), as well as in one case of chronic myelomonocytic leukemia (CMML). bcr negativity ruled out CML in 36 cases, confirming the morphologic diagnosis. In one case diagnosed as ET. bcr gene rearrangement was diagnostic of CML. The correct diagnosis made possible a different therapeutic approach in this young patient and resulted in cure after allogeneic bone marrow transplantation. The existence of such cases makes the use of molecular analysis essential in the evaluation of MPDs, even when the morphologic features do not unequivocally support the diagnosis of CML, as in this patient.
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PMID:bcr gene rearrangement analysis in myeloproliferative disorders other than chronic myelogenous leukemia. 881 85

We previously reported that serum granulocyte colony stimulating factor levels of chronic myelogenous leukemia patients in chronic phase (CP) are significantly lower than those of healthy persons or other hematological malignancies as assessed by chemiluminescence enzyme immunoassay (CLEIA). In this study, we clarify the difference in serum G-CSF levels between patients with primary myelofibrosis (PMF, myelofibrosis with myeloid metaplasia; MMM) and those with secondary myelofibrosis caused by several hematological disorders, using the same highly sensitive CLEIA method. It is clearly demonstrated that serum G-CSF levels of the patients with PMF and chronic neutrophilic leukemia (CNL) are extremely low, similar to those in patients with CML in CP in this study. From these data, it is speculated that the abnormal proliferation of granulocytes in PMF and CNL may not be due to the stimulation by G-CSF, and that a negative feedback mechanism might exist between peripheral granulocytes and serum G-CSF.
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PMID:Serum G-CSF levels in primary myelofibrosis and chronic neutrophilic leukemia as estimated by the highly sensitive chemiluminescence enzyme immunoassay (CLEIA). 888 66

Myeloproliferative diseases include primary polycythemia, primary thrombocytosis, primary myelofibrosis and chronic myeloid leukaemia. The average survival of patients with the former two diagnoses is more than 10 years, in the subsequent two it is only 5 years. Standard treatment of polycythemia still remains phlebotomy, only in patients with associated thrombocythemia and complications caused by it cytoreduction treatment is recommended (hydroxyurea, busulphan or interferon alpha). Patients with primary thrombocythemia due to hypofunctional thrombocytes suffer frequently from haemorrhagic and thrombotic complications. The objective of treatment is therefore to achieve a normal number of thrombocytes. Medicamentous procedures are the same as in primary polycythemia. In primary myelofibrosis and chronic myeloid leukaemia it is also necessary to reduce the pathological population in bone marrow by the above mentioned therapeutic procedures. Contrary to the former two diagnoses, the survival of patients with primary myelofibrosis is shorter and the patients need more supportive treatment. In chronic myeloid leukaemia also transplantation treatment can be used, if a suitable donor is available. The decision between classical and transplantation treatment must be made soon after establishment of the diagnosis. The objective of the following paper is to inform readers on the clinical course of different diseases, on differential diagnostic problems and changes in therapeutic procedure which have developed in recent years.
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PMID:[Myeloproliferative diseases]. 897 64

A considerable proportion of cases of myeloproliferative and lymphoproliferative disorders exhibit renal involvement. However, it is unclear whether the cytologic features, immunophenotype or grade of malignancy of the cells infiltrating the kidney differ from those of the primary tumor. This study was performed on 120 autopsy cases with the following diagnoses: acute myelogenous leukemia (AML, n = 22; subtypes M1 + M2, n = 12, subtype M4, n = 10), chronic myelogenous leukemia (CML, n = 7), agnogenic myeloid metaplasia/myelofibrosis (AMM/MF, n = 6), acute lymphocytic leukemia (ALL, n = 6), chronic lymphocytic leukemia (CLL, n = 9), other low-grade non-Hodgkin's lymphomas (low-grade NHL, n = 24), high-grade NHL (n = 21) and multiple myeloma (MM, n = 25). Renal involvement was investigated by light microscopy and immunohistochemistry. It was found in 34% of the cases, and was most common in ALL (83%) and low-grade NHL (50%) and least common in high-grade NHL (10%) and MM (12%). Dense infiltration of almost the entire kidney was most commonly seen in AML, low-grade NHL and ALL. Infiltration was bilateral and involved both the cortex and medulla in the majority of cases. When involvement of other organs was compared with that of the kidney, the lung was found to be involved in approximately the same number of cases, but liver involvement was more common and heart involvement less common. Reactive lymphocytic infiltration of the kidney was found in 18 of the 120 cases (15%), and was distinguished from scanty tumorous infiltration by immunohistochemical staining. No major phenotypical differences were found between the tumor cells infiltrating the kidney and those of the primary tumors in the bone marrow or lymph nodes. However, in one case of CML, the cells infiltrating the kidney were negative for KP1 and chloroacetate esterase, but could be identified by reactivity for CD34. The grade of malignancy in NHL was similar in both the nodal and renal manifestations.
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PMID:Renal involvement in myeloproliferative and lymphoproliferative disorders. A study of autopsy cases. 906 78


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