muramidase and Polycythemia-Vera

Topics: Anemia, Aplastic; Animals; Bacteriological Techniques; Bone Marrow; Glomerular Filtration Rate; Histological Techniques; Humans; Hypersplenism; Immunologic Techniques; Iodine Radioisotopes; Kidney; Leukemia, Lymphoid; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Muramidase; Neutrophils; Phosphorus Radioisotopes; Polycythemia Vera; Rabbits

Lysozyme, a myelomonocytic marker not only exerts bacteriolytic, but also immunomodulatoric properties and was found to bind to the glycosaminoglycan serglycin, an important constituent of the extracellular matrix (ECM). Pathological serum lysozyme levels were described in chronic myeloproliferative disorders (CMPDs) and other hematological conditions. In this context it is remarkable that in polycythemia rubra vera (PV), characterized by a proliferation particularly of the megakaryo- and erythropoiesis, serum lysozyme levels behave independently of the numbers of myelomonocytic cells in peripheral blood. To elucidate whether megakaryopoiesis might be the source of the increased serum lysozyme, we performed an experimental study on isolated and enriched megakaryocytes derived from bone marrow of patients with PV. Findings were compared to a group of patients with reactive (smoker's) polyglobuly (PG). In confirmation of previous results, quantification of serum lysozyme levels showed a slight elevation in the cohort of PV patients which was not correlated with the leukocyte count. Applying an immunohistochemical assay we were able to demonstrate intracytoplasmic lysozyme storage in megakaryocytes. Moreover, performing the reverse hemolytic plaque assay (RHPA), a technique which enables detection of secreted proteins at the single cell level, we found that 54% of the PV, but only 3% of the PG megakaryocytes spontaneously secreted lysozyme. After rhIL-3 treatment the secretion of lysozyme remained unchanged in PV but increased to 14% in PG. These findings suggest that the extent of megakaryocytic lysozyme secretion might discriminate PV from reactive conditions. Although a direct involvement of lysozyme in the regulation of aberrant megakaryopoiesis in PV is not likely, the results of the present study point to the possibility that lysozyme could be involved in the interactions of PV megakaryocytes with ECM. Moreover, the response to rhIL-3 significantly discriminates PV megakaryocytes from megakaryocytes of the PG group.

Topics: Female; Humans; Male; Megakaryocytes; Middle Aged; Muramidase; Polycythemia; Polycythemia Vera

Topics: Adult; Clinical Enzyme Tests; Hematologic Diseases; Humans; Leukemia, Monocytic, Acute; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Muramidase; Polycythemia Vera; Primary Myelofibrosis; Urine

Blood lysozyme estimation seems to be important in hematological practice. Serum levels are roughly proportional to the size of the pool and, above all, granulocytic renewal. Thus levels are increased compared with levels of circulating polynuclear cells. In bone marrow disorders, and particularly in myelofibrosis, owing to the infective granulopoiesis and/or increased destruction of the neutrophil polymorphs. It is lowered in neutropenia with a scanty bone marrow. It provides an important contribution to diagnosis of the type of acute leukemia, the fall in the lymphoblastic forms contrast with normal or increased levels in myeloblastic forms. Finally, there is a marked increase in lysosome urea in acute monocytic or myelomonocytic leukemia.

Topics: Anemia; Clinical Enzyme Tests; Granulocytes; Hematologic Diseases; Humans; Leukemia; Leukocyte Count; Muramidase; Myeloproliferative Disorders; Polycythemia Vera; Prognosis; Splenomegaly

Total reduced nicotinamide adenine dinucleotide (NADH) and reduced nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase activities were examined in human neutrophils. Approximately two-thirds of each enzyme activity was located in the granule fraction with the remainder in the soluble. The activities in a 27,000 x g supernatant from a sonic extract of human polymorphonuclear leukocytes were characterized. Both NADH and NADPH diaphorase were insensitive to cyanide and azide and showed greater activity at acid pH. K(m) values for nitroblue tetrazolium were not markedly different (33 muM with NADH and 12 muM with NADPH), but there was a 40-fold difference in K(m) for the reduced pyridine nucleotides (10 muM with NADH and 400 muM for NADPH). Since the intracellular concentration of both nucleotides is estimated to be about 50 muM, it is much more likely, from a kinetic argument, that the respiratory burst of phagocytosis is intiated by the oxidation of NADH rather than of NADPH.

Topics: Azides; Blood Proteins; Cell Separation; Cyanides; Glucuronidase; Humans; Lysosomes; Muramidase; NAD; NADH, NADPH Oxidoreductases; Neutrophils; Polycythemia Vera; Sonication; Tissue Extracts

Topics: Adult; Anemia; Anemia, Sideroblastic; Humans; Leukemia; Leukemia, Lymphoid; Leukemia, Monocytic, Acute; Leukemia, Myeloid; Leukemoid Reaction; Leukocytosis; Monocytes; Muramidase; Myeloproliferative Disorders; Polycythemia Vera; Primary Myelofibrosis

Topics: Agranulocytosis; Anemia, Pernicious; Cell Survival; Fluorine; Half-Life; Humans; Isoflurophate; Kinetics; Leukocyte Count; Lymphoma, Non-Hodgkin; Muramidase; Neutrophils; Polycythemia Vera; Radioisotopes

Topics: Adult; Aged; Blood Urea Nitrogen; Female; Humans; Hypokalemia; Kidney Tubules; Leukemia, Monocytic, Acute; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Leukocyte Count; Male; Middle Aged; Muramidase; Myeloproliferative Disorders; Polycythemia Vera; Primary Myelofibrosis; Radiography; Spleen

Topics: Adult; Anemia, Sideroblastic; Bone Marrow Diseases; Humans; Leukemia; Leukemia, Monocytic, Acute; Leukemia, Myeloid; Lymphoma; Monocytes; Multiple Myeloma; Muramidase; Polycythemia Vera; Primary Myelofibrosis

Topics: Adult; Electrophoresis; Glomerular Filtration Rate; Humans; Leukemia; Leukemia, Lymphoid; Leukemia, Myeloid; Leukocytes; Muramidase; Polycythemia; Polycythemia Vera

Topics: Aged; Female; Hemorrhagic Disorders; Humans; Muramidase; Polycythemia Vera

Topics: Agranulocytosis; Anemia, Aplastic; Anemia, Macrocytic; Bone Marrow; Chronic Disease; Folic Acid; Humans; Leukemia, Myeloid; Leukocyte Count; Leukocytes; Muramidase; Myeloproliferative Disorders; Neutrophils; Polycythemia Vera; Protein Binding; Vitamin B 12

Topics: Candida; Candidiasis; Hodgkin Disease; Humans; In Vitro Techniques; Leukemia; Leukocytes; Lymphoma; Multiple Myeloma; Muramidase; Neoplasms; Neutrophils; Peroxidases; Phagocytosis; Polycythemia Vera

Topics: Alkaline Phosphatase; Busulfan; Chlorambucil; Cyclophosphamide; Humans; Leukocyte Count; Muramidase; Phosphorus Isotopes; Polycythemia Vera; Uric Acid; Vitamin B 12

Topics: Adult; Anemia, Aplastic; Child; Hematologic Diseases; Hodgkin Disease; Humans; Leukemia; Leukemia, Erythroblastic, Acute; Leukemia, Lymphoid; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Leukemoid Reaction; Lymphoma; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Non-Hodgkin; Multiple Myeloma; Muramidase; Mycosis Fungoides; Myeloproliferative Disorders; Polycythemia Vera