acid-phosphatase and Thrombocytopenia

Topics: Acid Phosphatase; Adult; Bone Diseases; Cerebrosides; Epilepsy, Temporal Lobe; Gaucher Disease; Graft Rejection; Graft vs Host Reaction; Humans; Intellectual Disability; Kyphosis; Lymphopenia; Male; Postoperative Complications; Radionuclide Imaging; Spleen; Technetium; Thrombocytopenia; Transplantation, Homologous

One hundred forty-two patients with progressive, hormonally refractory advanced prostate carcinoma who had not received prior chemotherapy were randomized to receive either combination chemotherapy with 5-fluorouracil (5-FU), doxorubicin, and mitomycin C (FAM) or sequential chemotherapy with the same agents, i.e., mitomycin C, followed by doxorubicin on disease progression, followed by 5-FU. Objective tumor regressions were observed in 10 of 70 (14%) patients receiving the FAM treatment arm and 10 of 72 (14%) patients initially receiving mitomycin C. Of the 24 patients who received secondary therapy with doxorubicin alone, 3 (12.5%) achieved objective tumor regression. There were no responses among five patients who received tertiary therapy with 5-FU alone. The median survival time for all patients treated with the combination arm was 8.7 months, compared with 7.1 months for patients who received the FAM arm (P = 0.025). However, this modest survival advantage in favor of the FAM treatment arm must be weighed against significantly more myelosuppression experienced by these patients. The chemotherapeutic regimens used in this study have only minor clinical value in the treatment of hormonally refractory advanced prostate cancer.

Topics: Acid Phosphatase; Aged; Antineoplastic Combined Chemotherapy Protocols; Carcinoma; Doxorubicin; Fluorouracil; Humans; Leukopenia; Male; Mitomycin; Prostatic Neoplasms; Remission Induction; Survival Rate; Thrombocytopenia

A 63-year-old man who was incidentally found to have thrombocytopenia at a periodic physical examination visited our hospital. The spleen was palpable 3 finger-breadths below the navel level, and the liver was palpable 1 finger-breadth below the right costal margin. Peripheral blood examination showed WBC 2,900/microl, Hb 13.4 g/dl, and platelets 54 X 10(3)/ microl. Gaucher cells were recognized in the bone marrow by aspiration, and serum levels of total acid phosphatase and angiotensin converting enzyme were increased. Glucocerebrosidase activity was lower than the control level in bone marrow stroma cells, and modification of glucocerebrosidase genotype N188S was shown, which had been identified in the past. Furthermore, neurological examination was normal. Based on these results, we diagnosed the patient with Gaucher disease type I, and started enzyme replacement therapy. Gaucher disease is rare in Japanese, approximately 100 cases having been reported; diagnosis at older age is also relatively rare and, as far as we know, the oldest age reported in Japanese was 57 years old. Gaucher disease should be considered a differential diagnosis when thrombocytopenia and splenomegaly are found in elderly patients, although it is relatively rare.

Topics: Acid Phosphatase; Age of Onset; Biomarkers; Bone Marrow Cells; Diagnosis, Differential; Gaucher Disease; Glucosylceramidase; Humans; Male; Middle Aged; Mutation; Peptidyl-Dipeptidase A; Splenomegaly; Thrombocytopenia

Platelets from a mother and son with prolonged thrombocytopenia were shown in previous studies to contain giant organelles that developed in megakaryocytes and continued to evolve in circulating cells. Whole mount platelet preparations revealed that the large organelles were electron opaque like the serotonin-rich dense bodies in normal and patient platelets, and analytical electron microscopy revealed they contained large amounts of calcium and phosphorous in a ratio close to that found in normal platelet dense bodies. However, differences in physiology, biochemistry and morphology indicated the large opaque bodies and target-organelles in patient platelets were not aberrant dense bodies. The present study has shown that the giant organelles contain peroxidase activity like primary lysosomes in polymorphonuclear (PMN) leukocytes. Further, the giant organelles in patient platelets contain acid phosphatase activity. Analytical electron microscopy demonstrated that cerium, the capture ion for the acid phosphatase reaction product, was present in the opaque organelles with calcium and phosphorous, but not present in their normal dense bodies. Since normal sized lysosomes appeared to be reduced in patient platelets, it was concluded that the large structures were abnormal lysosomes, or fused with normal platelet lysosomes during their development. Similar giant lysosomes were not present in other patient blood cells. As a result the disorder can be considered a unique lysosomal disease of platelets.

Topics: Acid Phosphatase; Adult; Blood Platelets; Case-Control Studies; Cytoplasmic Granules; Family Health; Female; Humans; Infant, Newborn; Lysosomes; Male; Megakaryocytes; Microscopy, Electron; Peroxidase; Spectrum Analysis; Thrombocytopenia; X-Rays

Gaucher disease is the most frequent lysosomal storage disease and the most prevalent genetic disease among the Ashkenazi Jews (q approximately 0.047). The disease results from inherited defects of acid beta-glucosidase and the accumulation of the substrate, glucosylceramide, in cells of monocyte/macrophage origin. The therapeutic response to macrophage-targeted (alpha-mannosyl-terminated) alglucerase (Ceredase, at 60 to 15 IU/kg every 2 weeks) was analyzed in 33 patients (age range, 2 to 63 years; 15 splenectomized) with extensive Gaucher disease over periods of 6 to 24 months. The efficacy of several different doses and dosage reductions was evaluated. In patients with anemia (n = 30) and/or thrombocytopenia (n = 19), hemoglobin levels and platelet counts increased by 0% to 178% and 15% to 155%, respectively, within 3 to 12 months. In patients with splenomegaly (n = 17) and/or hepatomegaly (n = 28), liver and spleen volumes decreased in 6 months from 7% to 64% and 8% to 84% by 12 months, respectively. Hematologic and visceral improvements were noted at any doses between 60 and 15 IU/kg every 2 weeks. Furthermore, these positive initial therapeutic responses were persistent throughout therapy, with doses reduced by 50%. Pulmonary Gaucher disease did not improve clinically in 3 patients. Unrelated cirrhotic (n = 2), cholestatic (n = 1), or renal disease (n = 1) did not influence the rate of patient improvement. Two of five patients who developed serum antibodies against alglucerase during the first 6 to 12 months of therapy had mild antibody reactions. This study shows similar regression of clinical Gaucher disease manifestations with enzyme therapy, using doses between 30 and 60 IU/kg every 2 weeks. Therapeutic efficacy was not diminished after 50% to 75% dose reductions or in the presence of anti-enzyme antibodies.

Topics: Acid Phosphatase; Adolescent; Adult; Anemia; Bone Diseases; Child; Child, Preschool; Gaucher Disease; Glucosylceramidase; Hepatomegaly; Humans; Kidney Diseases; Liver Diseases; Lung Diseases; Middle Aged; Peptidyl-Dipeptidase A; Splenectomy; Splenomegaly; Thrombocytopenia

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.

Topics: Acid Phosphatase; Aged; Aged, 80 and over; Antigens, CD; Antigens, Differentiation; Antineoplastic Agents; B-Lymphocytes; Bone Marrow; CD11 Antigens; CD5 Antigens; Female; Histocytochemistry; Humans; Immunophenotyping; Leukemia, Hairy Cell; Leukemia, Lymphocytic, Chronic, B-Cell; Leukocytosis; Male; Middle Aged; Splenomegaly; Thrombocytopenia

In the work presented here we paid our attention to studying changes of the activity of two lysosomal enzymes-acid phosphatase and beta-glucuronidase in platelets after whole-body irradiation and application of certain clinically applied antifibrinolytics (Antilysin Spofa and epsilon-aminocaproic acid) on the activity of these enzymes in isolated platelets. From the results it is obvious that the activity of acid phosphatase and beta-glucuronidase increases in platelets of irradiated rats with a maximum on the 11th day after irradiation. In the same time interval after irradiation the most remarkable thrombocytopenia was observed. The application of Antilysin or EACA to intact, non-irradiated rats affects remarkably neither the activity of the followed enzymes nor the count of platelets. A statistically significant thrombocytopenia, in a comparison with controls was found in whole-body irradiated rats who received EACA or Antilysin. The postirradiation increase of the level of acid phosphatase in platelets exerts a similar character in rats who received EACA or Antilysin as in those who were only irradiated with a difference that the increase of the level of this enzyme was essentially lower in all the time intervals of interest. The beta-glucuronidase level in platelets of rats, who received EACA, was essentially unchanged as compared to controls. The application of Antilysin altered remarkably the level of beta-glucuronidase in platelets of whole-body irradiated rats in a comparison with a level of this enzyme in platelets of rats who received no Antilysin. Most remarkable changes were observed in latter time intervals (11th and 15th day) when the beta-glucuronidase level in platelets of rats after the application of Antilysin was remarkably higher than that in solely irradiated rats.

Topics: Acid Phosphatase; Aminocaproic Acid; Animals; Aprotinin; Blood Platelets; Glucuronidase; Male; Rats; Thrombocytopenia; Time Factors; Whole-Body Irradiation

The arachidonic acid metabolites thromboxane A2, a potent platelet aggregator, and prostacyclin, a potent vasodilator, are released early in endotoxin shock and may contribute to its pathologic sequelae. Plasma levels of thromboxane (Tx) A2 and prostacyclin were measured via radioimmunoassay of their stable metabolites immunoreactive (i) TxB2 and i6-keto-PGF1 alpha in tolerant and nontolerant rats after endotoxin. Long-Evans rats were made tolerant to endotoxin by four daily IV injections of S enteritidis (endotoxin) (0.1, 0.5, 1, and 5 mg/kg). In normal rats (N = 15) given LPS (IV, 15 mg/kg), only 11% survived at 24 h; in contrast, tolerant rats (N = 13) all survived even at a dose of 50 mg/kg. At 1 h, after endotoxin (15 mg/kg) IV, plasma i6-keto-PGF1 alpha in nontolerant rats was 1,005 +/- 149 pg/ml (N = 14) and continued to rise to 4,209 +/- 757 pg/ml (N = 5) (P less than 0.001) after 4 h. In tolerant rats, given endotoxin (15 mg/kg), plasma i6-keto-PGF1 alpha at 1 h was 800 +/- 203 pg/ml (N = 5) and was not significantly different (734 +/- 254 pg/ml) at 4 h. Plasma iTxB2 at both 1 and 4 h was significantly (P less than 0.01) lower in tolerant than nontolerant rats. Both iTxB2 and i6-keto-PGF1 alpha were significantly (P less than 0.01) lower in tolerant rats given 50 mg/kg IV endotoxin than nontolerant rats. Endotoxin-induced elevation in fibrin degradation products was significantly decreased (P less than 0.05) during endotoxin tolerance although there was no difference in the severity of thrombocytopenia. These composite observations demonstrate that endotoxin tolerance in the rat is associated with altered arachidonic acid metabolism.

Topics: Acid Phosphatase; Animals; Arachidonic Acid; Arachidonic Acids; Aspartate Aminotransferases; Blood Platelets; Disease Susceptibility; Fibrin Fibrinogen Degradation Products; Glucuronidase; Macrophages; Male; Prostaglandins F; Rats; Shock, Septic; Thrombocytopenia; Thromboxane B2

Bone marrow smears of 48 patients consisting of 12 normal cases, 36 patients with different haematological diseases-among them 9 cases of idiopathic thrombopenia, 4 cases of polycythaemia, and 9 cases of Hodgkin's disease - were examined cytochemically. Acid phosphatase, unspecific esterases, naphthol-AS-D-chloroacetate esterase, peroxydase, and leucin-aminopeptidase were represented; in addition the PAS reaction, fastgreen staining at pH 1.1, methyl-green pyronin staining and the lipid representation with Sudan black B were carried out. Besides those responses known from literature the different behaviour of acid megacaryocyte phosphatase in different haematological diseases must be particularly emphasized from all reactions.

Topics: Acid Phosphatase; Alkaline Phosphatase; Aminopeptidases; Blood Proteins; Esterases; Hematologic Diseases; Hodgkin Disease; Humans; Megakaryocytes; Peroxidases; Polycythemia Vera; Polysaccharides; Thrombocytopenia

On account of 2 own observations, main clinical and diagnostic features of Hairy cell leukemia (HCL) will be discussed. HCL is a rare, unusual type of chronic leukemia and is predominantly particular of middle-aged men. The occurrence of middle-sized lymphoid cells having a hairy cytoplasmic edge, and a tartrate-resistant acid PHOsphatase isoenzyme are the characteristic criteria of the HCL. The diffuse infiltration by hairy cells affecting primarily the spleen and bone marrow results in anaemia, granulocytopenia, thrombocytopenia and splenomegaly. Differential diagnosis have to be made in relation to other lymphatic leukemias, leukemic malignant lymphomas and monoclonal gammopathies as well as lymphotropic viral infections. Immunologic behaviour of hairy cells is like that of B-lymphocytes. Therefore, the term "leukemic reticuloendotheliosis" should no longer be applied.

Topics: Acid Phosphatase; Adult; Age Factors; Agranulocytosis; Anemia; B-Lymphocytes; Bone Marrow; Diagnosis, Differential; Humans; Leukemia; Leukemia, Myeloid; Lymphatic Diseases; Male; Middle Aged; Sex Factors; Spleen; Splenomegaly; Thrombocytopenia

Topics: Acid Phosphatase; Aged; Alkaline Phosphatase; Autopsy; Biopsy; Bone Marrow; Bone Marrow Examination; Esterases; Female; Histiocytes; Humans; Lymphatic Diseases; Microscopy, Phase-Contrast; Mitosis; Peroxidases; Thrombocytopenia

Acute promyelocytic leukaemia (A.P.L.) is a rare but important type of acute myeloid leukaemia characterized by major bleeding in association with thrombocytopenia, a specific peripheral blood and bone marrow picture, low plasma fibrinogen, and the presence in the serum of fibrin degradation products. These last abnormalities are related to the disseminated intravascular consumption of coagulation factors with secondary fibrinolysis. A.P.L. requires early recognition and urgent treatment. With optimal management up to half of the patients may achieve complete remission of two years or more. Undoubtedly patients with A.P.L. do especially well when treated in special centres and some patients with A.P.L. now die before the nature of their disease is recognized. Increased familiarity with the problem, which has been known for nearly 20 years, should yield great dividends for those few patients who have this disease.

Topics: Acid Phosphatase; Binding Sites; Bone Marrow Cells; Erythrocytes; Factor V; Factor VIII; Fibrinogen; Hematuria; Hemorrhage; Heparin; Humans; Leukemia, Myeloid; Leukocyte Count; Peroxidases; Platelet Transfusion; Prognosis; Prothrombin; Purpura; Remission, Spontaneous; Thrombocytopenia; Vitamin B 12

Topics: Acid Phosphatase; Adult; Antibiotics, Antineoplastic; Asparaginase; Blood Transfusion; Bone Marrow Examination; Daunorubicin; Drug Combinations; Esterases; Glucuronidase; Hematologic Diseases; Humans; Leukemia; Leukemia, Erythroblastic, Acute; Male; Megakaryocytes; Methotrexate; Peroxidases; Prednisolone; Spleen; Staining and Labeling; Thrombocytopenia; Vincristine

Topics: Acid Phosphatase; Blood Cell Count; Blood Coagulation Tests; Blood Platelets; Carcinoma; Clot Retraction; Depression, Chemical; Heparin; Humans; Male; Nitrophenols; Prostatic Neoplasms; Temperature; Thrombocytopenia

Topics: Acid Phosphatase; Adult; Biopsy; Blood Cell Count; Blood Coagulation Tests; Blood Protein Electrophoresis; Blood Sedimentation; Bone Marrow Cells; Chronic Disease; Clinical Enzyme Tests; Diagnosis, Differential; Gaucher Disease; Haptoglobins; Hemoglobinometry; Humans; Male; Osmotic Fragility; Splenomegaly; Thrombocytopenia

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; Anemia; Anemia, Aplastic; Blood; Humans; Purpura; Purpura, Thrombocytopenic; Thrombocytopenia