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 functional capacities of granulocytes in patients with chronic granulocytic leukemia are still a subject of controversy, probably due to the heterogeneity of the abnormalities observed from patient to patient. For a better definition of these abnormalities, 14 patients with untreated chronic granulocytic leukemia were studied. The patients were divided into three groups on the basis of the functional activities of their phagocytosing granulocytes. In four patients (group I), the granulocytes were normal in respect to particle ingestion, nitroblue tetrazolium (NBT)-stimulated reduction, cyanide-insensitive oxygen (O2) consumption, superoxide anion (O2-)-stimulated production, hydrogen peroxide (H2O2) production, and iodination. They also had a normal myeloperoxidase (MPO) content. In four patients (group III), the granulocytes were significantly defective in all of these activities. In the six remaining patients (group II), all the initial metabolic steps of the phagocytosing granulocytes (ingestion, NBT reduction, O2 consumption, O2-production, H2O2 production) were normal, as were the MPO content of the granulocytes, while iodination was strikingly decreased. These metabolic features suggested a degranulation defect which was observed ultrastructurally in the only patient studied among these six. The phagocytosing granulocytes of this patient did not degranulate and no deposits of MPO activity were seen in the phagosomes.
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PMID:Metabolic activity of phagocytosing granulocytes in chronic granulocytic leukemia: ultrastructural observation of a degranulation defect. 19 42

The rapid appearance of acute respiratory distress during the course of 25 hyperleukocytic leukemias was associated with the rapid increase of the leukocytosis. The regression of the tachypnea was spectacular when treating hyperleukocytosis by exchange transfusion and chemotherapy. Blood gas studies, although blurred to some extent by in vitro blast consumption of oxygen, showed a hypoxemia with a hypo-or normocapnia. The symptoms seem to be related to the leukostasis by the mechanical obstruction of the pulmonary capillaries. This leukostasis was shown to be responsible for a septal and alveolar oedema. The high frequency of this syndrome during the course of AGL and of acute phase of CGL seems to be linked to the low deformability of the myeloblasts. In CGL at its chronic phase, CLL or even in ALL, the absence of this syndrome could be explained by the greater deformability of the circulating cells. The hyperleukocytic AGL patients which do not have this syndrome are all characterized by a stable or slowly increasing leukocytosis. Thus, this syndrome seems to characterized by hyperleukocytic granulocytic leukemias with a rapid blood leukocyte doubling rate. Treatment in such cases is an emergency.
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PMID:Respiratory distress of hyperleukocytic granulocytic leukemias. 28 51

In a 49 year old man with blast crisis and massive leukocytosis due to chronic myelogenous leukemia, severe hypoxic respiratory failure developed despite a normal chest film. Correction of hypoxemia was observed after reduction of the white blood cell count by hydroxy-urea therapy. A similar episode occurred prior to death, and necropsy examination revealed extensive plugging of the pulmonary vasculature by leukemic blast cells but no infection or pulmonary edema. An inverse linear correlation was demonstrated between the peripheral white blood cell count and the efficiency of oxygen transfer in the lung as determined by the arterial to alveolar oxygen tension ratio. We postulate that mechanical obstruction and/or leukocyte mediated capillary endothelial injury caused the severe leukocyte mediated capillary endothelial injury caused the severe hypoxemia. Abnormalities of pulmonary gas exchange may be common in leukemic patients with markedly increased leukocyte counts.
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PMID:Reversible respiratory failure due to intravascular leukostasis in chronic myelogenous leukemia. Relationship of oxygen transfer to leukocyte count. 29 38

The initial rate of phagocytosis, oxygen consumption rate during phagocytosis, bactericidal capacity against Escherichia coli, and the granule protein composition of isolated mature-appearing granulocytes were studied in 23 patients with chronic granulocytic leukemia (CGL) with the simultaneous use of normal controls. The initial rate of phagocytosis was decreased (p less than 0.05) in the CGL patient group, as were oxygen consumption rate (p less than 0.001) and bactericidal capacity (p less than 0.001). Kinetic analysis of the ingestion rate showed CGL granulocytes to have the same capacity to bind the particles as normal granulocytes. Both specific and primary granule protein deficiencies were shown for CGL granulocytes, and these deficiencies were more pronounced at or near blast cell transformation. Analysis of all different granulocyte function parameters showed an inverse correlation to white blood cell counts (p less than 0.01) and to the percentage of immature granulocytes in peripheral blood (p less than 0.001). The leukocytosis doubling time was progressively shortened during the chronic course of the disease. A correlation was found between granulocyte function parameters and leukocytosis doubling time (p less than 0.001), indicating that granulocyte function was progressively deteriorating during chronic phase CGL, and may be an expression of increasing disturbance of the differentiation process.
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PMID:Granulocyte function in chronic granulocytic leukemia. II. Bactericidal capacity, phagocytic rate, oxygen consumption, and granule protein composition in isolated granulocytes. 106 28

The autoxidation of ascorbic acid (ASA) leads to the formation of compounds which are capable of glycating and crosslinking proteins in vitro. When the soluble crystallins from bovine lens were incubated with ASA in the presence of sodium cyanoborohydride, a single major adduct was observed, whose appearance correlated with the loss of lysine. When polylysine was reacted with equivalent amounts of ASA under the same conditions, this product represented half of the total lysine content after four weeks of incubation at 37 degrees C. This adduct was isolated and identified as N epsilon-(carboxymethyl)lysine (CML) by TLC, GC/MS and amino acid analysis. Several oxidation products of ASA were each reacted with polylysine in the presence of sodium cyanoborohydride to identify the reactive species. CML was the major adduct formed with either ASA and dehydroascorbic acid (DHA). Markedly diminished amounts were seen with L-2,3-diketogulonic acid (DKG), and L-threose, while no CML was formed with L-threo-pentos-2-ulose (L-xylosone). In the absence of sodium cyanoborohydride the yield of CML was similar with each of the ASA autoxidation products and required oxygen. Reactions with [1-14C]ASA gave rise to [14C]CML, but only with NaCNBH3 present. At least two routes of CML formation appear to be operating depending upon whether NaCNBH3 is present to reduce the putative Schiff base formed between lysine and DHA.
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PMID:The extent of N epsilon-(carboxymethyl)lysine formation in lens proteins and polylysine by the autoxidation products of ascorbic acid. 152 81

Ten patients with severe hematologic malignancies (four with acute leukemia, three with multiple myeloma, one with prolymphocytic leukemia, one with malignant lymphoma and one with blastic crisis of chronic myelogenous leukemia) developed respiratory failure during the period between April 1986 and May 1990. Clinically, the patients manifested high-fever, dyspnea refractory to oxygen therapy, diffuse pulmonary rales and severe hypoxemia without evidence of cardiogenic pulmonary edema. Chest roentgenograms displayed diffuse alveolar infiltrates. Respiratory failure occurred as early as 48 hours and as late as 66 days after the administration of intensive anti-neoplastic chemotherapy. At that time leukocyte count was between 100/microliters and 54,900/microliters. Marked leukocytosis was observed in two patients with AML and PLL. Respiratory failure was preceded by sepsis in one patient with AML and by pneumonia in nine patients. DIC was diagnosed in four patients. All patients treated with high dose methyl prednisolone (mPSL) within 12 hours after the onset of respiratory failure. Only one patient required assisted ventilation. High dose mPSL had significant effect on seven of ten patients. But three patients died from progressive respiratory failure, sepsis, pneumonia and multi-organ failure.
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PMID:[Clinical investigation on acute respiratory failure in patients with severe hematologic malignancy]. 194 22

Therapeutic leukapheresis of a 10-year-old girl with adult-type chronic myelocytic leukemia is described. The efficiency of WBC removal was noted to improve significantly after addition of hydroxyethyl starch to the anticoagulant infusion. In fact, the percentage of white cells removed more than doubled (39% vs. 16%) when comparing procedures of similar duration, with and without the sedimenting agent. Of particular interest was the relief of the patient's respiratory distress concomitant with the decline in her leukocyte count, a finding that has not previously been documented in pediatric patients. The arterial oxygen pressure remained greater than 95 mmHg after the procedure with hydroxyethyl starch compared with 70 mmHg after the procedures without it. In addition, her respiratory rate decreased from 70 on admission to the low 20s after the final procedure. Leukapheresis with hydroxyethyl starch was shown to be a safe procedure, and the use of hydroxyethyl starch was shown to be of particular benefit for prevention of prolonged apheresis procedures.
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PMID:Relief of respiratory distress with leukapheresis in a child with chronic myelocytic leukemia. 245 9

Decreased levels of neutrophils, Fc- and C3-expressing receptors, oxygen-dependent metabolism as well as antibody-dependent cytotoxicity of these cells to target cells were identified in advanced chronic myeloid leukemia, particularly, in blast crisis. Treatment with cytostatic agents resulted in further decrease in said parameters. However, treatment with prodigiozan was followed by improvement in cell functioning.
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PMID:[Effect of prodigiozan on the antibody-dependent cytotoxicity of neutrophils in chronic myeloid leukemia]. 252 67

The purpose of this study was to examine the effects of prior nonspecific immune stimulation (BCG), cross-reactive immunization (E coli J5 0111 whole cells [J5 WC], and core glycolipid [J5 CGL]), and type-specific immunization (Serratia marcescens core glycolipid [SM CGL]) on the cardiopulmonary variables and white blood cell counts of awake, monitored sheep following IV Serratia marcescens endotoxin. Comparison of cardiac output, pulmonary artery pressure, pulmonary capillary wedge pressure, pulmonary vascular resistance, alveolar-arterial oxygen gradients, and total white blood cells lead us to conclude that type-specific immunization (SM CGL) most effectively ameliorates the changes of gram-negative endotoxemia contrasted to nonimmunization. Core glycolipid cross-reactive (J5 CGL) immunization was somewhat more effective than whole-cell cross-reactive (J5 WC) immunization in this regard. Nonspecific immune stimulation (BCG) was able only to significantly decrease the changes in pulmonary vascular resistance compared to nonimmunization.
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PMID:Effect on cardiopulmonary changes of gram-negative endotoxemia in sheep after type-specific, cross-reactive, and nonspecific immune stimulation. 351 36

A rate of oxygen consumption by granulocytes was measured before cell stimulation and following their stimulation by zymosan in healthy persons and in patients with chronic myelocytic leukemia (CML). The rate of oxygen consumption by granulocytes of donors was higher than that of patients with CML. Addition of lysosomal acid soluble proteins, extracted from normal granulocytes, affected the respiratory activity of stimulated and non-stimulated both normal and leukemic cells, depending upon the protein concentration. The alterations were more marked in granulocytes of donors. Lysosomal acid soluble proteins, extracted from granulocytes of patients with CML did not change the cell respiratory activity.
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PMID:[Effect of lysosomal acid-soluble proteins on the respiratory activity of the granulocytes of healthy subjects and chronic myeloid leukemia patients]. 693 72


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