acid-phosphatase and Blood-Platelet-Disorders

Topics: Acid Phosphatase; Blood Platelet Disorders; Bone and Bones; Bone Diseases; Breast Neoplasms; Electrophoresis, Polyacrylamide Gel; Erythrocytes; Female; Hematologic Diseases; Humans; Isoenzymes; Leukemia; Leukocytes; Lipidoses; Male; Primary Myelofibrosis; Prostate; Prostatic Neoplasms; Spleen; Thromboembolism

The biochemistry of platelets from two unrelated patients with the gray platelet syndrome, a deficiency of platelet alpha-granules, has been evaluated. Ultrastructural studies of their platelets revealed the number of alpha-granules to be less than 15% of normal, whereas the number of dense bodies was within normal limits. Platelets from both patients had severe deficiencies of platelet factor 4 and beta-thromboglobulin (less than 10% of normal). Sodium dodecyl sulfate-polyacrylamide gel electrophoresis showed a marked deficiency of thrombin-sensitive protein in both patients. Analysis of the platelet-derived growth factor in one patient showed it was also markedly reduced. Levels of lysosomal enzymes, adenine nucleotides, serotonin, and catalase, and conversion of arachidonic acid by the lipoxygenase and cyclo-oxygenase enzymes, were within normal limits. The results provide important evidence to define the contents of alpha-granules and to differentiate these contents from the contents of lysosomal granules, dense bodies, and peroxisomes. Functional studies of these platelets showed deficiencies in ADP, thrombin, and collagen aggregation. The results suggest that alpha-granules or their contents make a contribution to normal platelet aggregation.

Topics: Acid Phosphatase; Adenine Nucleotides; Adult; Beta-Globulins; Blood Platelet Disorders; Blood Platelets; Cytoplasmic Granules; Female; Glucuronidase; Hexosaminidases; Humans; Infant; Male; Platelet Aggregation; Platelet Factor 4; Serotonin

Topics: Acid Phosphatase; Adenosine Diphosphate; Adenosine Kinase; Adenosine Triphosphate; Adenylate Kinase; Antigen-Antibody Complex; Blood Coagulation; Blood Coagulation Factors; Blood Platelet Disorders; Blood Platelets; Electron Transport Complex IV; Fibrinolysis; Hemostasis; Hexokinase; Humans; L-Lactate Dehydrogenase; Platelet Aggregation

The levels of four acid hydrolases, beta-N-acetyl glucosaminidase, beta-glucuronidase, beta-galactosidase, and acid phosphatase, and the extent of their release (release II) by thrombin was determined in platelets from nine normal subjects, nine patients with storage pool disease, and in normal platelets which had been exposed to aspirin. The levels of all four hydrolases were normal in patients with SPD. However, release of three of these hydrolases (acid phosphatase was an exception) by low concentrations of thrombin (0.015 and 0.04 U/ml) was decreased in the patients as a group, although considerable variation in the extent of release of each enzyme was noted. In contrast, aspirin failed to inhibit release II in normal platelets (except for a slight impairment in the release of beta-N-acetyl glucosaminidase), although release I (serotonin, ATP and ADP) was inhibited. All release defects could be overcome by using higher concentrations of thrombin (0.2 U/ml). The normal levels of acid hydrolases in the platelets of patients with SPD (who are deficient in the platelet dense granules) suggest that these enzymes are not normally stored in the dense granules, but rather in alpha-granules. The findings also support the conclusions of previous studies that the release reaction is impaired in SPD. This release defect appears to be different from that seen in normal platelets after exposure to aspirin.

Topics: Acetylglucosaminidase; Acid Phosphatase; Adenosine Diphosphate; Adenosine Triphosphate; Aspirin; Blood Platelet Disorders; Blood Platelets; Chromatography, Gel; Galactosidases; Glucuronidase; Humans; Hydrolases; Serotonin; Thrombin; Tritium

Topics: Acid Phosphatase; Adenine Nucleotides; Adenosine Diphosphate; Albinism; Aspirin; Blood Cell Count; Blood Platelet Disorders; Blood Platelets; Brain; Carbon Radioisotopes; Collagen; Factor X; Galactosidases; Glucuronidase; Heparin; Humans; Lysosomes; Microscopy, Phase-Contrast; Platelet Adhesiveness; Serotonin; Thrombin; Tissue Extracts; Venoms

Topics: Acid Phosphatase; Adenine Nucleotides; Adenosine Diphosphate; Aged; Blood Platelet Disorders; Blood Platelets; Carbon Radioisotopes; Clot Retraction; Collagen; Glucuronidase; Hexosaminidases; Humans; Male; Platelet Adhesiveness; Serotonin

Topics: Acid Phosphatase; Adenosine Diphosphate; Adenosine Triphosphate; Blood Coagulation Factors; Blood Platelet Disorders; Blood Platelets; Epinephrine; Hemostasis; Humans; Leukemia, Myeloid; Myeloproliferative Disorders; Platelet Adhesiveness

Topics: Acid Phosphatase; Adenosine Diphosphate; Aged; Aspirin; Blood Coagulation Factors; Blood Coagulation Tests; Blood Platelet Disorders; Collagen; Epinephrine; Fibrinogen; Glucuronidase; Heparin; Humans; Male; Platelet Adhesiveness; Thrombin

Topics: Acid Phosphatase; Blood Coagulation Factors; Blood Coagulation Tests; Blood Platelet Disorders; Blood Platelets; Glucose-6-Phosphatase; Glucosephosphate Dehydrogenase Deficiency; Glycogen; Hemorrhagic Disorders; Humans; Metabolic Diseases; Microscopy, Electron; Platelet Adhesiveness

Topics: Acid Phosphatase; Adenosine Diphosphate; Blood Coagulation Disorders; Blood Coagulation Factors; Blood Platelet Disorders; Blood Platelets; Clot Retraction; Factor V Deficiency; Factor X; Factor XI Deficiency; Humans; Hypoprothrombinemias; Lipoproteins; Osmotic Fragility; Phospholipids; Platelet Adhesiveness; Potassium; von Willebrand Diseases

Topics: Acid Phosphatase; Agammaglobulinemia; Aged; Anemia, Myelophthisic; Blood Platelet Disorders; Bone Marrow Diseases; Diethylstilbestrol; Erythrocytes; Erythropoiesis; Half-Life; Humans; Iron; Iron Isotopes; L-Lactate Dehydrogenase; Leukocytes; Male; Neoplasm Metastasis; Osmotic Fragility; Prostatic Neoplasms; Remission, Spontaneous; Time Factors

Topics: Acid Phosphatase; Blood Platelet Disorders; Blood Platelets; Hemostasis; Humans; Platelet Adhesiveness

Topics: Acid Phosphatase; Blood Coagulation Factors; Blood Platelet Disorders; Hemorrhage; Humans; Purpura, Thrombocytopenic

Topics: Acid Phosphatase; Blood Coagulation Disorders; Blood Platelet Disorders; Blood Platelets; Humans

Topics: Acid Phosphatase; Adenosine Triphosphate; Blood Platelet Disorders; Blood Platelets; Collagen; Epinephrine; Humans; Methods

Topics: Acid Phosphatase; Adenosine; Adenosine Diphosphate; Aminohydrolases; Anemia, Sideroblastic; Blood Platelet Disorders; Blood Platelets; Bolivia; Carbon Isotopes; Collagen; Environment; Epinephrine; Fibrinogen; Hemagglutination; Heparin Antagonists; Humans; Immune Sera; Megakaryocytes; Microscopy, Electron; Neuraminic Acids; Nucleotides; Peru; Platelet Adhesiveness; Purpura, Thrombocytopenic; Racial Groups; Thrombocytopenia; Uremia

Topics: Acid Phosphatase; Adult; Aged; Blood Platelet Disorders; Bone Diseases; Bone Neoplasms; Female; Gaucher Disease; Humans; Male; Middle Aged; Neoplasm Metastasis; Prostatic Neoplasms; Pulmonary Embolism

Topics: Acid Phosphatase; Blood; Blood Chemical Analysis; Blood Coagulation Disorders; Blood Platelet Disorders; Blood Platelets; Hodgkin Disease; Humans; Leukemia; Leukemia, Myeloid; Liver Cirrhosis; Plasma; Platelet Count; Potassium; Primary Myelofibrosis; Thrombocythemia, Essential