muramidase and Agranulocytosis

The discovery of cytosine arabinoside, and then the anthrocycline antibiotics, 6-thioguanine, vincristine, cyclophosphamide, and other drugs, has added to the armamentarium of known effective agents. The use of combination chemotherapy, the recognition of the need during induction for virtual marrow aplasia to obtain a remission, and recognition of the predilection of the disease for the central nervous system requiring prophylaxis constitute major advances. The impediment to long-term survival is the lack of effective maintenance therapy.

Topics: Acute Disease; Adolescent; Agranulocytosis; Antineoplastic Agents; Cells, Cultured; Child; Child, Preschool; Chromosome Aberrations; Chromosome Disorders; Disseminated Intravascular Coagulation; Drug Therapy, Combination; Female; Graft vs Host Reaction; Hodgkin Disease; Humans; Infections; Leukemia; Leukocytosis; Male; Muramidase; Preleukemia; Thrombocytopenia; Uric Acid

Topics: Acid Phosphatase; Agranulocytosis; Blood Bactericidal Activity; Chediak-Higashi Syndrome; Chemotaxis; Child, Preschool; Complement System Proteins; Cytoplasmic Granules; Female; Glucosephosphate Dehydrogenase Deficiency; Humans; Immunologic Deficiency Syndromes; Infant, Newborn; Infant, Premature; Leukocytes; Liver; Lysosomes; Macrophages; Male; Monocytes; Mononuclear Phagocyte System; Muramidase; NADH, NADPH Oxidoreductases; Neutrophils; Opsonin Proteins; Peroxidases; Phagocyte Bactericidal Dysfunction; Phagocytosis; Spleen

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

Cyclic neutropenia (CN) is an inherited disease known to occur in both humans and Gray Collie dogs. In dogs, the disease is characterized by a profound and cyclic decrease in circulating granulocytes at 12-day intervals. Other formed elements of the blood also show cyclic changes and thus the disease is also called cyclic hematopoiesis (CH). In this study, daily serum levels of lysozyme and a factor with macrophage migration inhibitory (MIF) activity were assayed. Both MIF activity and lysozyme levels were elevated more than 2-fold and fluctuated cyclically in CH dogs during the 12-day cycle: CH dogs CH 490 and CH 491, had 32.7 +/- 16.8% and 35.9 +/- 18.3% migration inhibitory activity, respectively, as compared to 15.1 +/- 1.4% in normal dog N 492; CH 490 and CH 491 had 78.7 +/- 43.7 units and 86.9 +/- 58.7 units of lysozyme, respectively, as compared to 33.4 +/- 1.7 units in N 492. The change of MIF activity tended to precede that of lysozyme activity in CH dogs. Furthermore, MIF levels and monocyte counts correlated significantly during the 12-day neutropenia cycle (CH 490, r = 0.677, P less than 0.001; CH 491, r = 0.583, P less than 0.01).

Topics: Agranulocytosis; Animals; Dogs; Hematopoiesis; Leukocyte Count; Macrophage Migration-Inhibitory Factors; Monocytes; Muramidase; Neutropenia; Neutrophils; Periodicity

Rabbit granulocyte lactoferrin, when infused into hamsters or rabbits, induces transient neutropenia, and in hamsters the lactoferrin promotes adherence of the granulocytes to the endothelial cell wall as monitored visually. In contrast, neither rabbit granule lysozyme nor human transferrin induces neutropenia in the rabbit nor does transferrin or bovine serum albumin affect the adherent properties in vivo of the phagocytic cells of the hamster. Thus lactoferrin enhances granulocyte adherence both in vivo and in vitro. It would appear that the promotion of margination of leukocytes by lactoferrin in vivo may contribute to the phenomenon of neutropenia during activation of granulocytes by chemotactic factors.

Topics: Agranulocytosis; Albumins; Animals; Cell Adhesion; Cell Aggregation; Chemotactic Factors; Cricetinae; Endothelium; Granulocytes; Infusions, Parenteral; Lactoferrin; Lactoglobulins; Leukocyte Count; Muramidase; Neutropenia; Rabbits; Transferrin

Unsaturated vitamin B12 binding capacity (UBBC) and serum lysozyme activity (LZM) were estimated durinng the endotoxin, prednisone and hydrocortisone marrow granulocyte reserve (MGR) pool tests. Our results showed, that no additional mechanism except the shift of MGR from marrow caused granulocytosis after typhoid vaccine administration. While the prednisone, when given orally diminished additionally the number of the physiologically destroyed neutrophils. The hydrocortisone, however, showed the results very similar to those obtained after typhoid vaccine adminstration. Thus the hydrocortisone test seems to be most useful. It gives as good information as typhoid vaccine test but does not show its side-effects.

Topics: Agranulocytosis; Bone Marrow Cells; Humans; Hydrocortisone; Muramidase; Prednisone; Typhoid-Paratyphoid Vaccines; Vitamin B 12

Topics: Adult; Agranulocytosis; Child; Humans; Infant; Leukemia; Muramidase; Neutropenia

Current research on the mechanism of action of the IUD has focused on a local, low-grade endometritis in preventing blastocystic implantation. With rabbit antisera to rat neutrophils, a neutropenic state was induced in rats having a silk suture in one uterine horn. Assay for nidation sites in the severely polymorphdepleted rats revealed no implantation sites in the IUD horn and an average of 5.8 sites in the control horn, suggesting that inflammation plays a relatively minor role, if any, in the mechanism of action of the IUD.

Topics: Agranulocytosis; Animals; Embryo Implantation; Female; Immune Sera; Intrauterine Devices; Leukocyte Count; Muramidase; Neutropenia; Neutrophils; Pregnancy; Rats; Uterus; Vaginal Smears

The prednisone test revealed normal bone marrow reserve of neutrophils (BMR) in subjects with innocent neutropenias e.g. neutropenias not associated with any other disturbances in haemopoiesis or with increased incidence of infections, assumed to be an individual anomaly of the subject. On the other hand, diminution or absence of MBR in chronic hypoplastic neutropenias, transient post-chemotherapeutic neutropenias and neutropenias with increased destruction of neutrophils were observed. The magnitude of that diminution was not dependent of the cause of neutropenia. It is suggested, that the prednisone test (and probably tests with other BMR mobilizing agents) may serve as screening tests for exclusion (or confirmation) of relative granulopoietic insufficiency as the cause of neutropenia. Only after confirmation of reduced BMR another explanations of neutropenia should be sought using other method as muramidase level, adrenaline test or granulocyte kinetics with DF32P, 3HDFP, or 51Cr.

Topics: Adolescent; Adult; Aged; Agranulocytosis; Antineoplastic Agents; Blood Cells; Bone Marrow Cells; Diagnosis, Differential; Female; Humans; Male; Methods; Middle Aged; Muramidase; Neutropenia; Prednisone; Time Factors

Topics: Adult; Aged; Agranulocytosis; Arthritis, Rheumatoid; Blood Volume; Cell Survival; Felty Syndrome; Female; Half-Life; Humans; Isoflurophate; Leukocyte Count; Male; Middle Aged; Muramidase; Neutropenia; Phosphorus Radioisotopes; Splenectomy

Topics: Acute Disease; Age Factors; Aged; Agranulocytosis; Anemia; Bone Marrow Examination; Cell Transformation, Neoplastic; Ecchymosis; Erythropoiesis; Female; Hematocrit; Humans; Iron; Leukemia; Leukemia, Monocytic, Acute; Leukemia, Myeloid; Leukocytes; Male; Middle Aged; Muramidase; Myeloproliferative Disorders; Splenomegaly; Syndrome; Thrombocytopenia

Topics: Aged; Agranulocytosis; Bone Marrow; Bone Marrow Cells; Cell Survival; Child; Creatinine; Cyclophosphamide; Female; Humans; Leukemia, Myeloid; Leukocyte Count; Male; Melphalan; Methotrexate; Middle Aged; Muramidase; Neutrophils; Phosphorus Isotopes; Time Factors; Vinblastine

Human cyclic neutropenia is characterized by severe depression of blood neutrophil levels approximately every 21 days. To investigate the mechanism of cyclic neutropenia four patients were studied with daily complete blood counts, serial bone marrow examinations, marrow reserve testing, serum muramidase determinations, DF(22)P granulocytokinetic studies, and, in one patient, in vivo [(3)H]TdR labeling. Periodogram analysis of the serial blood counts in the latter patient and visual inspection of multiple cycles in the others revealed periodic fluctuations in the levels of blood neutrophils, monocytes, lymphocytes, reticulocytes, and platelets. Rhythmic changes in the morphologic and radioisotopic studies as well as the marrow reserve tests and muramidase measurements were consonant with a mechanism of periodic failure of marrow production rather than peripheral destruction. Human cyclic neutropenia is analogous to cyclic neutropenia in the grey collie dog and may be viewed as the consequence of cyclic hematopoiesis.

Topics: Agranulocytosis; Animals; Blood Cell Count; Blood Platelets; Bone Marrow Examination; Dogs; Erythrocyte Count; Hematopoiesis; Humans; Isoflurophate; Leukocyte Count; Muramidase; Periodicity; Phosphorus Radioisotopes; Thymidine; Tritium

Topics: Adolescent; Adult; Aged; Agranulocytosis; Bone Marrow; Cell Survival; Felty Syndrome; Female; Fluorine; Humans; Isoflurophate; Kinetics; Lymphoma, Non-Hodgkin; Male; Middle Aged; Muramidase; Neutrophils; Radioisotopes; Splenomegaly

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

Topics: Agranulocytosis; Antibodies, Anti-Idiotypic; Blood Chemical Analysis; Erythrocytes; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Immunoglobulins; Immunologic Deficiency Syndromes; Infant; Karyotyping; Leukocyte Count; Leukocytes; Male; Monocytes; Muramidase; Neutrophils; Phagocytosis; Protein Binding; Rheumatoid Factor; Saliva; Vitamin B 12

Topics: Acid Phosphatase; Adult; Agranulocytosis; Alkaline Phosphatase; Bacterial Infections; Chediak-Higashi Syndrome; Chemotaxis; Female; Glucuronidase; Humans; Leukocytes; Lysosomes; Male; Muramidase; Peroxidases; Skin Window Technique; Staphylococcal Infections; Streptococcal Infections

Topics: Absorption; Adult; Agglutination Tests; Agranulocytosis; Antibody Formation; Antibody Specificity; Chromatography, DEAE-Cellulose; Female; Fetomaternal Transfusion; Fluorescent Antibody Technique; Humans; Immunoglobulin G; Infant, Newborn; Isoantibodies; Maternal-Fetal Exchange; Muramidase; Neutrophils; Pregnancy; Pyoderma

Topics: Agranulocytosis; Bone Marrow; Bone Marrow Cells; Bone Marrow Examination; Humans; Leukocyte Count; Muramidase; Neutrophils

Topics: Agranulocytosis; Animals; Bone Marrow; Bone Marrow Cells; Kidney; Leukocytes; Lung; Male; Mechlorethamine; Muramidase; Rats; Spleen; Time Factors

Topics: Adolescent; Adult; Agranulocytosis; Alkaline Phosphatase; Female; Humans; Leukocytes; Metabolism, Inborn Errors; Muramidase; Pedigree

12 grey collie dogs had cyclic neutropenia with the neutropenia recurring at 11.8+/-0.1-day intervals. The recovery from neutropenia was accompanied by a single wave of myeloid proliferation, an increase in marrow myeloid-labeling indices, and an increase in serum muramidase levels. After recovery from neutropenia during the period when blood neutrophils (PMN) were normal or increased, marrow myeloid precursors became scarce. The decline in marrow precursors and marrow PMN reserves heralded the recurrence of neutropenia. Neither diisopropyl fluorophosphate (DF(32)P) leukokinetic studies nor the rate of development of neutropenia suggested shortened PMN survival as a mechanism for the neutropenia. These studies indicate that the cyclic neutropenia is due to a regularly recurring failure in PMN production.

Topics: Agranulocytosis; Animals; Autoradiography; Dog Diseases; Dogs; Female; Hematopoiesis; Hematopoietic Stem Cells; Leukocyte Count; Male; Muramidase; Neutrophils; Periodicity; Thymidine; Tritium

Topics: Adult; Agranulocytosis; Alpha-Globulins; Beta-Globulins; Blood Cell Count; Blood Protein Electrophoresis; Bone Marrow Examination; Cobalt Isotopes; Cytarabine; Humans; Leukemia, Monocytic, Acute; Leukemia, Myeloid, Acute; Male; Muramidase; Periodicity; Protein Binding; Vitamin B 12

The kinetics of blood neutrophils was investigated by means of the in vitro radioactive diisopropyl fluorophosphate method in 35 patients with a chronic, steady-state neutropenia. There were 17 patients in whom the half disappearance time of neutrophils was normal. In 10 of these patients, the production of neutrophils was low and in 7, production was normal. In 18 patients the half disappearance time of neutrophilic granulocytes was shorter than normal. The production of neutrophilic granulocytes was low in five of these patients, normal in eight patients, and increased in five. An attempt was made to correlate other laboratory measurements with the kinetic picture, but no relationship was found; the marrow neutrophil reserve as measured by endotoxin or cortisol injection; marrow cellularity on aspiration or biopsy; in vitro-labeling index with (3)HTdR; or serum lysozyme concentration proved of no value in identifying the various kinetic groups. The only finding that seemed to correlate with the kinetic picture was the presence or absence of splenomegaly. In 12 of the 18 patients with a short half disappearance time, splenomegaly was present whereas in 15 of 17 patients with a normal half disappearance time, there was no splenomegaly. Of 20 patients with greater than 1000 neutrophils per mm(3), 17 were found to have a normal total-blood neutrophil pool. Thus these patients, with many of their cells marginated, agree to have a "shift neutropenia."Myelocyte to blood transit time and myelocyte generation time, as measured in seven patients by in vivo labeling with diisopropy fluorophosphate, proved to be essentially normal. Thus, it appears that in chronic neutropenia, increased or decreased production of neutrophils is accomplished by increasing or decreasing early precursor input into the system.

Topics: Agranulocytosis; Bone Marrow; Bone Marrow Cells; Bone Marrow Examination; Cell Survival; Chronic Disease; DNA; Endotoxins; Fluorine; Hematopoiesis; Humans; Hydrocortisone; Isoflurophate; Leukocyte Count; Muramidase; Neutrophils; Radioisotopes; Splenomegaly; Thymidine; Time Factors; Tritium; Uric Acid

Topics: Adolescent; Adult; Aged; Agranulocytosis; Allopurinol; Anti-Bacterial Agents; Antibiotics, Antineoplastic; Blood Transfusion; Cytarabine; Humans; Leukemia; Leukemia, Erythroblastic, Acute; Leukemia, Lymphoid; Leukemia, Myeloid; Leukemia, Myeloid, Acute; Mercaptopurine; Methotrexate; Middle Aged; Muramidase; Nausea; Prednisolone; Thrombocytopenia; Vincristine

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: Adolescent; Adult; Aged; Agranulocytosis; Bone Marrow; Female; Humans; Leukocyte Count; Male; Middle Aged; Monocytes; Muramidase; Neutrophils; Splenectomy

Topics: Adolescent; Adult; Agranulocytosis; Diagnosis, Differential; Female; Humans; Male; Middle Aged; Muramidase

Topics: Agranulocytosis; Humans; Leukocytes; Muramidase

Topics: Agranulocytosis; Humans; Leukocytes; Muramidase

Topics: Adult; Aged; Agranulocytosis; Bone Marrow; Female; Humans; Kinetics; L-Lactate Dehydrogenase; Leukocyte Count; Male; Middle Aged; Muramidase

Topics: Agranulocytosis; Immune Sera; Leukocytes; Leukopenia; Mechlorethamine; Muramidase; Time Factors

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: Adult; Agranulocytosis; Anemia, Macrocytic; Anemia, Pernicious; Clinical Enzyme Tests; Folic Acid Deficiency; Humans; Muramidase; Vitamin B 12