muramidase and Hematologic-Diseases

Topics: Hematologic Diseases; Humans; Inflammatory Bowel Diseases; Muramidase

Topics: Actins; Animals; Bone Marrow; Bone Marrow Cells; Cell Differentiation; Cell Membrane; Cell Nucleus; Cytoplasmic Granules; Endoplasmic Reticulum; Golgi Apparatus; Hematologic Diseases; Hematopoiesis; Histocytochemistry; Humans; Hydrolases; Inflammation; Leukemia; Muramidase; Myosins; Neutrophils; Peroxidase; Peroxidases

Topics: Aneuploidy; Basophils; Blood Platelets; Cell Transformation, Neoplastic; Child; Clinical Enzyme Tests; Cytogenetics; Eosinophilia; Fetal Hemoglobin; Fever; Hematologic Diseases; Humans; Leukemia, Myeloid; Leukocyte Count; Lymphatic Diseases; Muramidase; Primary Myelofibrosis; Prognosis; Skin Manifestations; Thrombocytosis; Vitamin B 12

Topics: Agranulocytosis; Anemia, Aplastic; Blood Cell Count; Clinical Trials as Topic; Hematologic Diseases; Humans; Leukemia; Leukocytes; Lymphocytes; Methods; Monocytes; Muramidase; Myeloproliferative Disorders

We describe a patient whose peripheral blood neutrophils and bone marrow precursors (beyond promyelocytes) contained multiple large azurophilic granules. There were also giant granules in eosinophils, basophils, melanocytes, renal tubules, thyroid, and neurones, but not lymphocytes or monocytes. His clinical course included recurrent (ultimately fatal) infections and severe neurologic impairment. Immunofluorescent staining with fluoroscein- and rhodamine-conjugated antisera to primary and secondary granule markers showed virtually all of the granulocyte granules and rare monocyte granules to be fusion products containing both markers. Electron microscopy showed the granules to be large peroxidase-containing lysosomes. Only rare normal primary and secondary granules were present. Superoxide generation in response to opsonized zymosan was 7.3 nmole/min/10(6) cells (control 8.9); but in response to phorbol myristate acetate, only 2.2 (control 9.4). Nitroblue tetrazolium slides showed 3+ dye reduction in response to opsonized zymosan by 90% of granulocytes (control 91%) and to phorbol myristate acetate by 22% (control 99%), with 71% producing only a minimal 1+ response. Cellular contents of myeloperoxidase and beta-glucuronidase were elevated, but the percent release during exocytic degranulation was equivalent to control. Ingestion of complement-opsonized Staphylococcus aureus and zymosan was also normal. Killing of Staphylococcus aureus was 60% at 90-min incubation (control 92%). Granulocyte cyclic adenosine monophosphate (AMP) content was 4 pmole/10(7) cells (control 3.1). In order to determine whether these characteristics derived from the cells' genetic program or their environment, the patient's bone marrow was grown in long-term culture. Granulocytes produced in vitro demonstrated the same morphology, same defect in activation of nitroblue tetrazolium reduction, and same normal cyclic AMP level as those harvested from peripheral blood. These studies describe a new disorder of granulocytes; the structural similarity to, but biochemical differences from, Chediak-Higashi disease indicate the probable heterogeneity of mechanisms for the same morphological abnormality.

Topics: Abnormalities, Multiple; Bone Marrow Cells; Cells, Cultured; Cyclic AMP; Cytoplasmic Granules; Hematologic Diseases; Humans; Infant, Newborn; Lactoferrin; Male; Muramidase; Neutrophils; Periodic Acid-Schiff Reaction; Peroxidase

A simple method for quantifying lysozyme in serum and urine has been established by modifying the procedures of sample application in the lysoplate method reported by Osserman and Lawlor [1]. A strip of filter paper is partially immersed in a liquid sample, dried at room temperature and cut into discs which are later placed on the surface of an agarose gel containing Micrococcus lysodeikticus. The follow-ng procedures for determination are carried out as described in the lysoplate method. There was no statistically significant loss of enzyme activity during the sample preparation. Lysozyme dried on filter paper is so stable that it can be stored at room temperature for at least 13 weeks and can be mailed. The sensitivity of the method is increased by pretreatment of the filter paper with Triton X-100 and consequently corresponds to that of the lysoplate method. The reproducibility of our method is practically good since the variation coefficient of the diameters of lytic zones within assays is around 1%. There is a very close correlation between lysozyme levels determined by this method and by the lysoplate method with serum samples obtained from patients with various hematological diseases (r = +0.994). The method can be utilized for routine clinical determinations of lysozyme.

Topics: Drug Stability; Hematologic Diseases; Humans; Immunodiffusion; Muramidase; Paper; Polyethylene Glycols; Specimen Handling; Time Factors

Topics: Hematologic Diseases; Humans; Leukemia, Lymphoid; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Lymphoma; Muramidase; Myeloproliferative Disorders

Lysozyme activity was studied in blood smears, serum, and urine of patients suffering from leukaemia or other haematological diseases. Increased enzyme activity was found in myelocytic, myelomonocytic and monocytic leukaemia and equally in secondary granulocytosis and polycythaemia vera. Reduced rates were found in lymphocytie leukaemia, malignant lymphoma with bone marrow involvement, and myelophthisic conditions. A rise in urinary lysozyme occurred when the serum level exceeded 50 microgram/ml. Abundant activities were found in myelomonocytic and monocytic leukaemias. Using the bacteriolytic method in blood smears, no enzyme activity was demonstrated in cells of acute or chronic lymphocytic leukaemia, in monocytic leukaemia however, almost all cells show strong reaction. In acute myelocytic or myelomonocytic leukaemia, the portion of positive cells changes from case to case depending on the degree of cell differentiation and maturation. In chronic myelocytic leukaemia there was no difference as compared to enzyme activity of myelocytes in bone marrow of control cases. Thus the bacteriolytic demonstration of lysozyme in blood smears may additionally contribute to distinction of different types of blastic leukaemias, and serum lysozyme also may allow more reliable insight into granulocytic and monocytic myelopoiesis than morphologic studies of blood or bone marrow smears can do, e.g. in agranulocytosis and pancytopenia.

Topics: Anemia, Aplastic; Hematologic Diseases; Humans; Leukemia, Erythroblastic, Acute; Leukemia, Lymphoid; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Leukocytes; Lymphoma, Large B-Cell, Diffuse; Lymphoma, Non-Hodgkin; Muramidase; Waldenstrom Macroglobulinemia

Topics: Acute Disease; Adolescent; Child; Child, Preschool; Hematologic Diseases; Hemophilia A; Humans; Leukemia; Male; Muramidase

Topics: Amino Acid Sequence; Animals; Dogs; Hematologic Diseases; Humans; Kidney Diseases; Molecular Weight; Muramidase; Rats

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

Topics: Bacteriolysis; Cell Wall; Granulocytes; Hematologic Diseases; Humans; Leukocytes; Molecular Weight; Monocytes; Muramidase; Neutrophils

Topics: Adult; Female; Hematologic Diseases; Humans; L-Lactate Dehydrogenase; Leukemia; Leukemia, Lymphoid; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Leukocyte Count; Lymphocyte Activation; Male; Muramidase; Remission, Spontaneous; Uric Acid

Topics: Female; Hematologic Diseases; Humans; Leukemia; Lymphoma; Male; Muramidase

Topics: Adult; Female; Hematologic Diseases; Humans; Hypokalemia; Leukemia; Leukemia, Myeloid; Male; Muramidase

Topics: Hematologic Diseases; Humans; Iodine Isotopes; Kidney Diseases; Muramidase

Topics: Bone Marrow Examination; Hematologic Diseases; Humans; Leukemia; Leukemia, Myeloid; Leukocyte Count; Muramidase; Time Factors

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

Topics: Albinism; Animals; Bone Marrow Examination; Hematologic Diseases; Histocytochemistry; Leukocyte Count; Leukocytes; Light; Lymphocytes; Lysosomes; Mice; Muramidase; Spleen

Topics: Adolescent; Adult; Agranulocytosis; Albinism; Anemia, Hemolytic; Blood Cell Count; Blood Cells; Blood Platelets; Bone Marrow Cells; Child; Etiocholanolone; Hematologic Diseases; Humans; Hypersplenism; Kinetics; Leukocyte Count; Leukocytes; Light; Lymphocytes; Male; Monocytes; Muramidase; Prednisone; Splenomegaly; Thrombocytopenia

Topics: Acid Phosphatase; Animal Diseases; Animals; Carnivora; Cathepsins; Cattle; Cattle Diseases; Escherichia coli; Glucuronidase; Hematologic Diseases; Leukocytes; Lymphocytes; Lysosomes; Microscopy, Phase-Contrast; Muramidase; Neutrophils; Salmonella typhimurium; Streptococcus pyogenes

Topics: Adult; Anti-Infective Agents, Local; Carcinoma; Hematologic Diseases; Humans; Lymphatic Diseases; Muramidase; Neoplasms