Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this report we present the autopsy findings of acute megakaryoblastic leukemia with tumor formation in a 2-year-old female infant with Down's syndrome. Chromosomal analysis of blast cells revealed constitutional anomaly of trisomy 21 and two other related types of abnormal clones. Flow cytometric examination revealed blast cells expressing Ia-like or HLA-DR antigens. Postmortem examination showed extensive infiltration of leukemic cells in most of the examined organs, including the bone marrow with myelofibrosis. Tumor masses in the maxillary, frontal and femoral bones and the atria of the heart had undergone massive infiltration of atypical blast cells with an increase in the reticulin network. The final diagnosis was confirmed by ultrastructural cytochemistry of the platelet peroxidase reaction as well as by immunological staining utilizing anti-platelet glycoprotein IIb/IIIa, antiplatelet factor 4 and anti-beta-thromboglobulin antibodies for the blast cells. It seems likely that platelet-derived growth factor, secondary to an increase in the reticulin network, plays a major role in myelofibrosis of acute megakaryoblastic leukemia with tumor formation.
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PMID:Acute leukemia of megakaryocyte lineage with tumor formation. An autopsy case of patient with Down's syndrome. 296 53

The plasma concentration of beta-thromboglobulin was serially measured in nine patients with septicemia, ten patients with pneumonia and five thrombo- and granulocytopenic patients with acute leukemia. Six patients with septicemia out of the eight studied on days 1-3 and all eight patients studied 7-14 days after onset had an abnormal high beta-thromboglobulin level. One patient with pneumonia out of six studied on days 1-3 and six out of nine studied on 7-14 days after onset had an abnormal high value. A rising trend in plasma beta-thromboglobulin with the highest mean levels at one to two weeks after onset was common to both groups. Positive ethanol gelation, increased level of fibrin/fibrinogen degradation products, decreased antithrombin III, increased FVIII complex and disproportionate ratio of FVIII:C to FVIIIR:Ag were common in both groups in the early stages of the disease. All the five patients with leukemia had a lower than normal beta-thromboglobulin level throughout the study but showed in the coagulation parameters changes similar to those observed in the other groups. Judging from the commonness of abnormal beta-thromboglobulin values in the two first patient groups, low grade platelet activation is a normal response in severe infection.
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PMID:Plasma beta-thromboglobulin in severe infection. 618 May 2

An antiserum to beta-thromboglobulin was used in immunofluorescence to detect normal and malignant megakaryocytes. In normal peripheral blood and bone marrow smears, only platelets and megakaryocytes were specifically stained by this antiserum. Among 25 cases of acute lymphoid and non-lymphoid leukaemia, only 2 exhibited a clear positivity in a % of blasts, thus proving their megakaryocytic origin. This method is proposed as a simple and useful tool to detect normal and malignant megakaryocytes on smears.
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PMID:Detection of normal and malignant megakaryocytes by anti beta-thromboglobulin serum. An immunofluorescence study. 618 32

In 9 patients with myeloproliferative diseases (MPD) (6 with myelofibrosis, MF, 1 with Ph1 positive chronic granulocytic leukaemia, CGL, 1 with primary eosinophilia, PE, 1 with pre-leukaemia syndrome, preL) collagen, epinephrine, and ADP-induced aggregation, N-ethylmaleimide-induced malondialdehyde (MDA) production, beta-thromboglobulin (beta-TG) plasma levels, and platelet turnover were studied. Collagen-induced aggregation was found to be normal in 7 patients, absent in 1, and reduced in 1. In all but 3 patients, aggregation with ADP was markedly reduced. Epinephrine-induced aggregation was decreased in 7 patients. No difference was found between mean MDA production in MPD (3.21 +/- 0.50 nmol/10(9) PLTs) and in control group of 21 normal subjects (3.04 +/- 0.26 nmol/10(9) PLTs). Mean beta-TG levels were significantly higher (P less than 0.01) in MPD patients (165.00 +/- 28.29 ng/ml) than in healthy controls (81.76 +/- 14.63 ng/ml). Mean platelet production half-time was significantly shorter in MPD (2.48 +/- 0.24 d) than in the control group (3.43 +/- 0.17 d), after adjustment for age by covariance analysis (P less than 0.005). Our data do not indicate an abnormal prostaglandin synthesis and are consistent with the hypothesis that a disseminated intravascular platelet aggregation might take place in MPD patients.
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PMID:Defective platelet aggregation and increased platelet turnover in patients with myelofibrosis and other myeloproliferative diseases. 646 Oct 58

In a child with acute megakaryoblastic leukemia--severe thrombocytopenia and myelofibrosis, EM studies on bone marrow showed a strict topographic relationship between the presence of clusters of abnormal megakaryocytes and the increased number of fibroblasts and extracellular fibers. Megakaryocytes and platelets lacked alpha-granules while the plasma thromboglobulin level was three times the normal level. This suggested that the alpha-granular proteins were synthesized but not retained in alpha-granules. If this occurs, the increased marrow levels of platelet-derived growth factor and factor 4 would favor the proliferation of fibroblasts and the synthesis of collagen, and thereby promote myelofibrosis. After therapy-induced remission, the number of marrow megakaryocytes decreased, the alpha-granules were normally produced, the plasma beta-thromboglobulin level was normal and the myelofibrosis disappeared. These observations suggest that during acute megakaryoblastic leukemia, an acquired gray-platelet syndrome occurs and that the local excretion of alpha-granule proteins triggers the myelofibrosis.
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PMID:Myelofibrosis and acute megakaryoblastic leukemia in a child: topographic relationship between fibroblasts and megakaryocytes with an alpha-granule defect. 646 1

A series of 101 consecutive patients with chronic myeloproliferative disorders including polycythaemia vera, chronic myelogenous leukaemia, idiopathic myelofibrosis and essential thrombocythaemia have been studied. The aim was to establish the incidence of thrombotic and haemorrhagic complications and the possible role played by platelet number and function. The total incidence of haemostatic complications was 21% and the platelet functional tests investigated (platelet aggregation, generation of malondialdehyde, endogenous serotonin, beta-thromboglobulin and platelet coagulant activity) were of little help for predicting these clinical complications.
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PMID:Thrombohaemorrhagic complications in 101 cases of myeloproliferative disorders: relationship to platelet number and function. 658 Jan 71

A growth factor was found in the supernatants of MOLT-4f, a cell line derived from acute T lymphoblastic leukemia. This factor, which we designated leukemia-derived growth factor from MOLT-4f (LDGF-M4), is different from interleukin 2. LDGF-M4 has features of a polypeptide with a molecular weight in the range of 5,000-15,000, as indicated by gel diffusion chromatography. LDGF-M4 does stimulate MOLT-4f and at least two other T cell lines that do not respond to interleukin 2. Because MOLT-4f cells produce and respond to LDGF-M4, this factor may contribute to the independence of MOLT-4f and related T leukemia cell lines.
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PMID:Leukemia-derived growth factor (non-interleukin 2) produced by a human malignant T lymphoid cell line. 698 93

Stimulation of human platelets to release results in decreased buoyant density. This decreased density provides a tool to detect circulating platelets which have participated in a thrombotic process. Platelet density gradient centrifugation using Stractan was standardized and the effects of anticoagulation, temperature, and osmolarity were investigated. In 7 out of 32 patients with thrombotic disease less dense platelets were found. Platelet activation in the patient group was also indicated by spontaneous aggregation (10/32), decreased circulating platelet aggregate ratios (5/24) and elevated plasma beta-thromboglobulin levels (2/ 11). Several of these tests were also abnormal in diabetes mellitus thrombocytosis, leukaemia and several systemic diseases with thrombotic complications. The platelet density test using Stractan is reproducible and independent of other tests for platelet activation and is therefore potentially a useful extension of platelet function testing in patients with thrombotic disease.
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PMID:Determination of the density distribution of human platelets--methodological aspects and comparison with other tests for platelet activation. 711 98

Although the exact mechanism for the progression of myelofibrosis in acute megakaryoblastic leukaemia is unclear, certain humoral factors released from the proliferating megakaryoblasts that are unable to store these factors in their defective alpha-granules, including platelet derived growth factor (PDGF), fibroblast growth factors (FGF), platelet factor-4 (PF-4), transforming growth factor-beta (TGF-beta) and beta-thromboglobulin, could result in increased collagen synthesis by bone marrow fibroblasts. Recently, the human megakaryoblastic leukaemia cell line MEG-01 has been shown to produce both TGF-beta and PF-4 which have enhanced the growth of bone marrow fibroblasts. Therefore, we have examined the presence of a fibroblast growth stimulating activity and the humoral factors that might be responsible for it in the supernatant of the human megakaryoblastic leukaemia cell line ELF-153 recently established in our laboratory from a patient with acute myelofibrosis. A new fibroblast growth stimulating activity has been identified in the supernatant of the ELF-153 human megakaryoblastic leukaemia cell line that is independent of the percentage of fetal calf serum in NRK-49F fibroblast agar clonogenic assays and is not due to any of the known fibroblast growth stimulating humoral factors including PDGF, epithelial growth factor, TGF-alpha or beta, tumour necrosis factor-alpha, interleukin-1, 2, 4 or 6, FGF, fibronectin, PF-4 and factor VIII AG. Also, in vivo, subcutaneous injection of ELF-153 megakaryoblastic leukaemia cells into nude mice formed, in three out of the five mice after 6 weeks, subcutaneous tumours with a very rigid texture whose histological examination revealed dense infiltration by blast cells and pronounced reticular fibrosis. Immunohistochemistry demonstrated exclusive deposition of collagen III in the extracellular matrix whereas laminin and collagen IV were absent.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:A new fibroblast growth stimulating activity from the human megakaryoblastic leukaemia cell line ELF-153: in vitro and in vivo findings. 748 50

The myelosuppressive effects of human chemokines were evaluated in vitro on normal myeloid progenitors obtained from bone marrow and cord blood, on bone marrow progenitors from patients with acute or chronic leukemia, on proliferation of human factor-dependent cell line M07e, and in vivo on myelopoiesis in mice. Preincubation of human MIP-1 alpha, MIP-2 alpha, interleukin (IL)-8, platelet factor (PF) 4, monocyte chemotactic and activating factor (MCAF), and interferon-inducible protein-10 (IP-10) in an acetonitrile (ACN) solution significantly enhanced the specific activity of these chemokines for in vitro suppression of granulocyte-macrophage (CFU-GM), erythroid (BFU-E), and multipotential (CFU-GEMM) progenitor cells stimulated to proliferate with a colony stimulating factor plus steel factor (SLF). Combinations of any two of these ACN-treated chemokines synergized to suppress colony formation of CFU-GM, BFU-E, and CFU-GEMM at chemokine concentrations below that at which combinations of non-ACN treated chemokines are active. Cord blood progenitors, as previously reported, were in a slow or noncycling state and nonresponsive to inhibition by chemokines. However, after suspension culture with GM-CSF, IL-3, and SLF, they were placed into rapid cell cycle and were responsive to inhibition by ACN-treated chemokines. Low doses of these ACN-pretreated chemokines were active in vivo in suppressing absolute numbers and cycling status of femoral marrow CFU-GM, BFU-E, and CFU-GEMM in C3H/HeJ mice. Other chemokines, alone and in combination, including MIP-1 beta, MIP-2 beta, GRO-alpha NAP-2, and RANTES, were inactive in vitro and in vivo whether or not they were pretreated with ACN. While heterogeneity in responsiveness of CFU-GM from different patients with leukemia to suppression by ACN-treated chemokines was apparent, if the patients had CFU-GM responsive to one of the active chemokines these cells were responsive to the other active chemokines; if patient CFU-GM were not responsive to one of the chemokines, they were not responsive to the other active chemokines. M07e colony-forming cells were responsive to the growth-inhibiting effects of the active ACN-treated chemokines, alone and in combination, but these effects were rapidly reversible and sustained only by multiple daily additions of chemokines. These results should be of value in considering these chemokines for potential clinical use and for assessment of their mechanisms of action, alone and in combination.
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PMID:Human chemokines: enhancement of specific activity and effects in vitro on normal and leukemic progenitors and a factor-dependent cell line and in vivo in mice. 749 26


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