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)

Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked staining reactions with antibodies against type III procollagen (pN collagen), type IV collagen, fragment P1 of laminin and factor VIII. Patients with osteomyelosclerosis had particularly increased collagen content, including both newly deposited type III collagen (pN collagen) and mature collagen fibres. As in normal bone marrow, argyrophilic fibres and type III collagen displayed a close co-distribution, which was also demonstrated for type IV collagen and laminin. While normal bone marrow sinusoids had discontinuous basement membranes, fibrosing bone marrow was characterized by endothelial cell proliferation and capillarization, with the development of continuous sheets of basement membrane material beneath endothelial cells.
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PMID:Bone marrow stroma in idiopathic myelofibrosis and other haematological diseases. An immunohistochemical study. 200 Dec 83

Using the megakaryocytic leukemia cell lines, K-562 and CMK established from a Down's patient with acute megakaryoblastic leukemia, we studied the changes of antigen expression, cytosolic Ca2+ mobilization, thromboxane (TX) A2 formation and gene expression during megakaryocyte differentiation. We found that thrombospondin synthesis and platelet factor (PF)-4 gene expression were specific for mature megakaryoblasts, whereas collagen unresponsiveness and prostaglandin E1-induced Ca2+ mobilization were noted in immature megakaryoblasts alone. This experiment shows that functional and genetic analysis are useful for characterizing the leukemic megakaryoblastic cells. We analyzed the clinical, hematologic and genetic features of 4 patients with M7, and acute megakaryoblastic transformation of CML, MDS and essential thrombocythemia. In two patients, prednisolone and 6-MP were effective in cytoreduction. In 3 patients with increased platelet counts, normal CFU-Meg formation, the megakaryoblasts with platelet production, or the coexistence of immature megakaryoblasts with mature megakaryocytes were observed, thus indicating that some megakaryoblastic leukemia cells still have the capacity of differentiation. One patient had megakaryoblastic cells with PF-4 gene expression. These clinical findings suggest that the megakaryoblastic leukemia could not be characterized as usual leukemia and a more sensitive marker is required to differentiate leukemic megakaryoblasts from normal megakaryoblasts.
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PMID:[Megakaryocytic leukemia cell lines and megakaryocytic leukemia]. 238 Oct 77

Although collagen myelofibrosis indicates poor prognosis in late stages of chronic myelogenous leukemia, the significance of reticulin stain-measured fibrosis in newly diagnosed patients is unknown. One hundred and thirty-eight patients with untreated or minimally treated chronic phase Philadelphia chromosome-positive chronic myelogenous leukemia had reticulin stain studies made on their bone marrows at diagnosis. Reticulin fibrosis was graded on a scale of 1 to 4. Significant (Grade 3 or 4) fibrosis was noted in 65 patients (47%). Compared with patients with mild (Grade 1 to 2) reticulin fibrosis, those with significant fibrosis had a higher incidence of splenomegaly greater than or equal to 10 cm (29% versus 49%; P = 0.02), hemoglobin less than 10 g/dl (19% versus 49%; P less than 0.01), weight loss greater than or equal to 6.75 kg (10% versus 30%; P = 0.11), marrow blasts greater than or equal to 5% (7% versus 28%; P less than 0.01), peripheral blasts greater than or equal to 3% (30% versus 46%; P = 0.09), and additional karyotypic abnormalities (1% versus 17%; P less than 0.01). The incidence of thrombocytosis was similar in the two groups. Prognostically, median survival was significantly shorter for the 26 patients with Grade 4, compared with the 39 patients with Grade 3, and the 73 patients with Grade 1 or 2 reticulin fibrosis (32 versus 49 versus 57 months; P = 0.03). Reticulin fibrosis is a useful biologic and prognostic index in newly diagnosed patients with chronic phase chronic myelogenous leukemia.
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PMID:The relevance of reticulin stain-measured fibrosis at diagnosis in chronic myelogenous leukemia. 243 99

In 100 patients with nonblastic Ph1-positive chronic granulocytic leukemia (CGL) the main features from the initial bone marrow biopsy were correlated with other clinical and hematological parameters and analyzed for prognostic significance. The mean number (+/- SD) of megakaryocytes per square millimeter of marrow tissue was 25.9 +/- 18.8. Marked reticulin fibrosis was observed in 19% of the patients but in only 5 cases it was diffuse, whereas collagen deposition was found in 10 patients. Reticulin fibrosis correlated significantly with white blood cell count and peripheral blood blast cell percentage, but not with platelet count. Actuarial survival studies demonstrated that marked diffuse reticulin fibrosis (p = 0.0005) and more than 15 megakaryocytes/mm3 of marrow tissue (p = 0.01) were associated with a poorer prognosis. However, they lost their prognostic influence when included in a multivariate regression model together with the patients' relative risk according to the currently most widely accepted CGL prognostic system. Such results indicate that bone marrow biopsy features add little prognostic weight to known prognostic indicators in CGL.
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PMID:Prognostic evaluation of initial bone marrow histopathological features in chronic granulocytic leukemia. 250 69

In order to review the histological characteristics and to evaluate the presence of valuable findings for diagnostic or prognostic purposes, 80 patients diagnosed of chronic myeloproliferative syndromes (CMPS) who had undergone initial bone marrow biopsy were studied in retrospect. The patients were distributed into 26 cases of chronic myeloid leukaemia (CML), 22 of myelofibrosis (MF), 16 of polycythaemia vera (PV) and 16 of essential thrombocythaemia (ET). The histological findings in the 26 cases of CML consisted of hypercellular bone marrow with fat depletion in all cases; marked thickening of trabeculae was seen in 4 cases and neoformation-like osseous tissue in 7 others. Erythroblastic nests were found in 11 patients, eosinophilia in 23 instances, and abnormal location of immature precursor cells (ALIP) was present in 16 cases. Reticulinic fibrosis was found in 22 patients, collagen fibrosis in 6 and sinusoidal dilatation in 5 cases. Of the 22 MF patients, 8 had normal bone trabeculae, while 14 had trabecular thickening, of moderate degree in 6 cases and marked in 8. Osteoid tissue formation was observed in 21 instances; diminished or absent fat was appreciated in 19 cases. Erythroblastic nests were present in 12 instances, ALIP in 5 and eosinophilia in 14. Reticulin fibres were increased in 22 instances, collagen fibrosis was present in 18 cases, sinusoid dilatation in 12, and lymphoid follicles were seen in 4 patients. Trabecular thickening was found in 15 cases of the PV group (16 patients) and osteoid tissue formation in 2. Fatty tissue was decreased or absent in 11 instances Erythroblastic nests plus eosinophilia were seen in 15 cases, and ALIP in 2.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[The value of the initial bone biopsy in chronic myeloproliferative syndromes. Review of 80 cases]. 266 69

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

The maintenance of hemopoietic precursors in long-term liquid bone marrow cultures (LTBMC) is associated with the presence of an adherent stromal layer composed of heterogeneous cell populations. We have used a culture assay to promote the growth of one of its cellular components and characterize its properties. Freshly obtained bone marrow cells and cells derived from the adherent layer of LTBMC were grown in methylcellulose-clotted plasma in the presence of phytohemagglutinin-stimulated leukocyte-conditioned medium (PHA-LCM), hydrocortisone (HC), and citrated normal human plasma. Both sources contained cells (CFU-RF) that gave rise to colonies of cells with a reticulofibroblastoid appearance. In the presence of HC, most colonies contained lipid-laden cells. Colonies could be further propagated as adherent layers when transferred into liquid cultures. These cells produced laminin, fibronectin, and collagen types I, III, IV, and V. They were negative for Von Willebrand factor VIII. The ability to synthesize laminin and collagen type IV distinguished these cells from a population of previously described bone marrow fibroblasts (CFU-F). The relationship of CFU-RF to hemopoietic precursors was investigated using patients with chronic myeloid leukemia and bone marrow transplant recipients. Cells within CFU-RF-derived colonies were uniformly negative for the Philadelphia chromosome, thus making it unlikely that they belonged to the malignant hemopoietic clone. CFU-RF-derived colonies in bone marrow transplant recipients were found to be exclusively of host origin. Both observations support the view that CFU-RF is not part of the repertoire of hemopoietic stem cells.
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PMID:Characterization of reticulofibroblastoid colonies (CFU-RF) derived from bone marrow and long-term marrow culture monolayers. 308 97

Collagen synthesis stimulated by materials released from platelets (MRFP) of patients with chronic granulocytic leukemia (CGL) and polycythemia vera (PV) was measured in human bone marrow fibroblasts derived from normal individuals. The MRFP of patients with CGL and PV stimulated normal fibroblast collagen synthesis significantly compared to the response from normal MRFP. Platelets derived from patients with CGL and PV may have some factors that dramatically stimulate collagen synthesis which is relevant to the frequently observed myelofibrosis in these diseases.
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PMID:Increased collagen synthesis of bone marrow fibroblasts by materials released from platelets of chronic granulocytic leukemia and polycythemia vera. 308 91

Atrophie blanche is a syndrome of painful vasculitic infarctive lesions of the lower extremities that heal leaving characteristic atrophic, porcelain white scars. The syndrome may occur as an idiopathic entity or associated with various hematologic and collagen vascular disorders. We present the first recognized case of atrophie blanche in a patient with chronic myelogenous leukemia. This presentation may be part of the expanding spectrum of nonspecific cutaneous manifestations of leukemia (leukemids).
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PMID:Atrophie blanche in chronic myelogenous leukemia. 316 50

Platelet function was evaluated in 20 patients with chronic myelocytic leukemia (CML), all Ph positive. Seven showed abnormal epinephrine-induced aggregation, while four had impaired both ADP- and collagen-induced aggregation. The platelets of all patients aggregated with arachidonic acid, thus ruling out cyclooxygenase or lipoxygenase deficiency. The intracellular concentrations of ATP and ADP were significantly below normal, and the ratio of ATP/ADP was greater than normal in all 12 patients. ATP released from platelets by Lumi-aggregometer was reduced. In four patients with abnormal ristocetin-induced aggregation, vWF:Ag, RCoF, and FVIII:C were all reduced. No significant inactivation of factor VIII was induced in normal plasma by incubation with patient's plasma. The crossed immunoelectrophoretic analysis revealed that vWF:Ag in these patients was mainly composed of more anodic component as compared with that of normal plasma. The ratio of vWF:Ag/RCoF was significantly greater than normal. A marked increase of factor VIII and a rapid return of vWF:Ag and RCoF to the baseline after the 1-deamino-8-arginine vasopressin (DDAVP) infusion were observed. Transient increase in vWF:Ag after the infusion of DDAVP appeared with less anodic forms and in the same relative proportion as that in normal plasma. The present study shows that in some patients with CML storage pool disease occurs with acquired von Willebrand disease.
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PMID:Acquired von Willebrand disease and storage pool disease in chronic myelocytic leukemia. 348 75


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