Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Variable numbers of foamy cells (macrophages with foamy cytoplasm) were noted in generalized organs from four patients who received repeated were noted in generalized organs from four patients who received repeated platelet transfusions. The underlying disease in three cases was aplastic anemia, and the remaining case was chronic myelocytic leukemia. In two patients (aplastic anemia and chronic myelocytic leukemia) bone marrow transplantation (BMT) was done. Opportunistic infection was noted in three out of four cases. The foamy cells were stained black with Sudan black B. Variable amounts of materials immunoreactive with antihuman platelet antibody were demonstrated in most of the foamy cells. Ultrastructurally, the foamy cells contained myelin-like materials. The foamy cells described here resembled those demonstrable in the spleen from patients with idiopathic thrombocytopenic purpura. We suggest that the foamy appearance of the macrophage results from incomplete intracellular degradation of phagocytosed platelets.
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PMID:Foamy cell syndrome associated with repeated platelet transfusions. 354 14

The anatomy and pathology of the splenic red pulp was studied in three-dimensional reconstructions of methylmethacrylate embedded blocks of tissue obtained after splenectomy, as well as by morphometrical analysis of a large number of specimens. The sinuses of the spleen form a plexus of anastomosing vessels with remarkable buds. Capillaries end as sheathed capillaries in the cord tissue, the 'filtering' area, but a large proportion of the red pulp cords appear to be 'non-filtering'. These might form part of the lymphatic compartment, which is separate from the white pulp and its extension along the capillaries. This area has not yet been described in man. The change in the volume and structure of the various components of the red pulp were studied in 60 controls and in cases of traumatic rupture, idiopathic thrombocytopenic purpura, aplastic anaemia, autoimmune haemolytic anaemia, congenital spherocytosis, splenic congestion, and Hodgkin's disease. Significant differences were found in the volume of filtering and non-filtering areas, the size of the sinus compartment, and the degree of vascularization; these differences were only partially expected, for instance in disorders with excessive erythrocyte sequestration. A decrease of the 'non-filtering' area in Hodgkin's disease might indicate an unknown aspect of this disease. In agreement with our previous paper on the amount of white pulp, spleens removed because of traumatic rupture and those incidentally removed during abdominal surgery may not be combined as a single control group, because of significant and probably functional differences in the composition also of the red pulp.
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PMID:The splenic red pulp; a histomorphometrical study in splenectomy specimens embedded in methylmethacrylate. 400 86

By using a combination of a heterologous antiserum to GPIb/glycocalicin and a radiolabeled monoclonal antibody to GPIb/glycocalicin, we were able to develop a sensitive and specific radioimmunoelectrophoretic assay that can distinguish small amounts of glycocalicin from GPIb. Normal plasmas were found to contain glycocalicin, even in samples treated with protease inhibitors and centrifuged extensively to remove platelets and platelet fragments. Confirmation that the plasma antigen had a relative molecular weight similar or identical to glycocalicin was obtained from studies employing gel chromatography and affinity chromatography. An immunoradiometric assay was developed to quantify plasma glycocalicin, and normal plasma was found to contain approximately 1-3 micrograms/ml. The plasma of a patient with severe thrombocytopenia due to aplastic anemia had less than 12.5% of the normal level of glycocalicin, whereas two patients with thrombocytopenia due to diseases of increased platelet destruction (idiopathic thrombocytopenic purpura and hemolytic-uremic syndrome) had normal levels. Thus, there appears to be ongoing catabolism of platelet GPIb in vivo, and we postulate that the plasma level of glycocalicin reflects a complex function of factors, including platelet count, platelet turnover, and the site of platelet destruction.
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PMID:Evidence that glycocalicin circulates in normal plasma. 623 8

Because of frequent unavailability of HLA-compatible platelets or their inefficiency in increasing platelet counts in patients requiring platelet transfusions, platelet refractoriness remains a major problem. Since human gamma globulin has been shown to interfere with the binding of platelet-reactive immunoglobulin G (IgG) to platelets in vitro, and since gamma globulin has proved effective in reversing destruction of platelets in idiopathic thrombocytopenic purpura, we decided to investigate intravenous gamma globulin as an adjunct to random-donor platelet transfusions. We studied three patients, one with acute T-cell leukemia, and two with aplastic anemia. When a total dose of 2 g/kg of body weight of intravenous gamma globulin was infused over five to six days in these patients, two of them showed a remarkable increment in platelet counts and improved hemostasis with random-donor platelet transfusions. We conclude that intravenous gamma globulin may be used in critical situations to improve the response of patients refractory to random-donor platelet transfusions.
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PMID:Improved response of patients refractory to random-donor platelet transfusions by intravenous gamma globulin. 642 52

Using procedures that were effective in the purification of human urinary erythropoietin (Epo), we attempted initial purification of megakaryocyte colony-stimulating factors (CSF) in urinary extracts from patients with aplastic anemia (AA) and idiopathic thrombocytopenic purpura (ITP). Comparison of colony stimulation by purified human Epo and crude urinary extracts revealed: (1) that the pure Epo augments megakaryocyte colony formation in culture and (2) MEG-CSF activity is also present in materials other than Epo in the crude urinary extracts from the two types of patients. Similar to purification of Epo, ethanol precipitation and sulfopropyl-Sephadex chromatography provided twofold and threefold increases in the specific activity of MEG-CSF, respectively. In contrast to Epo, however, significant inactivation of MEG-CSF activity was seen with phenol treatment. The elution profile of MEG-CSF seen on hydroxylapatite chromatography of urinary extracts was different from that of Epo. These data provided a basis for initial steps for purification of MEG-CSF and support the notion that MEG-CSF is distinct from Epo.
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PMID:Characterization of human megakaryocyte colony-stimulating factor in the urinary extracts from patients with aplastic anemia and idiopathic thrombocytopenic purpura. 660 Jun 33

A new immunoglobulin (Ig) for intravenous use was given in high doses to 4 children with refractory idiopathic thrombocytopenic purpura (ITP) and 2 children with idiopathic aplastic anemia (IAA). Within 5-10 days after initiation of Ig therapy the platelets of the children with ITP rose to 300,000-650,000/mm3 and could be maintained at normal levels with one Ig infusion every 1-3 weeks. No reaction was observed in the 2 patients with IAA.
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PMID:High-dose intravenous gammaglobulin therapy of refractory, in particular idiopathic thrombocytopenia in childhood. 678 58

In order to measure platelet-associated IgG (PAIgG), we devised a solid-phase enzyme immunoassay employing a competitive binding of peroxidase-conjugated anti-IgG antiserum between platelets and polystyrene tubes coated with IgG. The amounts of peroxidase bound to the tubes were measured in a spectrophotometer by an enzymatic reaction. This method is highly sensitive, reproducible and can be carried out more simply. the PAIgG values of normal controls averaged 21.6 +/- 6.6 (SD) ng/10(7) platelets. 27 (93%) of 29 patients with idiopathic thrombocytopenic purpura (ITP), who had a platelet count of less than 15 X 10(4)/microliter, had PAIgG values greater than those of controls by 2 SD and averaged 205.5 +/- 323 ng. There was a significant inverse correlation between platelet count and PAIgG value of ITP patients. the PAIgG values of patients with aplastic anemia were within normal range.
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PMID:Quantification of platelet-associated IgG with competitive solid-phase enzyme immunoassay. 680 Jan 91

Platelet volume was measured in 20 healthy children, 18 patients with acute idiopathic thrombocytopenic purpura (ITP), 24 patients with chronic ITP, 22 patients with aplastic anemia, and 17 patients with acute leukemia in childhood. The authors compared the platelet volume of these patients by use of peak platelet volume (PPV or mode of the platelet volume), mean platelet volume (MPV) and the percent of large platelets (PLP) as parameters of the platelet volume, and then studied the relationship between the platelet volume and the specific gravity of platelets from these patients. The following was the platelet volume of normal children: PPV was 6.7 +/- 1.1 micrometer 3, MPV was 9.9 +/- 1.6 micrometer 3, PLP was 26.0 +/- 7.7%. All parameters of the platelet volume were remarkably large in chronic ITP, but only PPV was small in acute ITP. On the other hand, all parameters were small in both aplastic anemia and acute leukemia. In acute ITP and chronic ITP, the platelet volume returned to normal value after these thrombocytopenias disappeared. The platelet volume, however, still remained small even after remission in acute leukemia. There was better correlation between MPV and PLP than PPV and PLP. The density analysis of the platelets obtained from platelet-rich plasma (PRP) revealed that PLP in each medium (1.010 approximately 1.052) of normal PRP increased slowly in proportion to the increase of specific density of the media. On the contrary, most of large platelets obtained from the patients with acute ITP and chronic ITP were especially contained in the medium of high specific density (1.052). Large platelets of aplastic anemia and acute leukemia, however, were found in both low and high density media. The authors believe that analysis of the platelet volume using above mentioned three parameters would provide good weapon in early differential diagnosos of acute ITP, chronic ITP, and aplastic anemia. And these findings also may suggest the difference of thrombopoiesis between acute ITP and chronic ITP.
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PMID:Differential diagnosis of various thrombocytopenias in childhood by analysis of platelet volume. 692 90

The urinary extract from patients with severe, chronic idiopathic thrombocytopenic purpura (ITP) was capable of inducing significant thrombocytosis in rats in vivo and enhancing megakaryocyte colony formation in culture of mouse bone marrow cells. The apparent specific activity of megakaryocyte colony stimulating factor (MEG--CSF) in the ITP extract was approximately one-half of that of the urinary extract from patients with aplastic anaemia (AA). Daily injections of ITP extract did not cause an increase in Hb concentration, while rats receiving AA urinary extract revealed profound erythropoiesis 3 weeks later. In vitro assay of erythropoietin (EPO) failed to show significant EPO activity in the extract from patients with ITP. These findings excluded the possibility that the observed activities of thrombopoiesis-stimulating factor (TSF) and MEG--CSF in the ITP urinary extract are due to contaminating EPO. Urine of patients with severe ITP appears to be a good source of TSF and MEG--CSF.
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PMID:Thrombopoiesis- and megakaryocyte colony-stimulating factors in the urine of patients with idiopathic thrombocytopenic purpura. 697 2

Megakaryocyte colon-stimulating factor (MEG-CSF) in the urinary extracts from patients wit aplastic anaemia (AA) revealed two distinct peaks of activity on Sephadex G-200 gel filtration with apparent molecular weights of 155,000 and 76,000. Both fractions induced significant thrombocytosis in peripheral blood and megakaryocytosis in the spleen of rats. Heterogeneity of MEG-CSF was also found in the extracts from the urine of patients with idiopathic thrombocytopenic purpura. The higher molecular weight MEG-CSF was significantly reduced when the gel filtration was performed under the dissociating conditions. Ion-exchange chromatography indicated that the higher molecular weight MEG-CSF had a different charge from the lower molecular weight MEG-CSF. These results suggest that the apparent heterogeneity of MEG-CSF is due to interaction of MEG-CSF with other proteins in the urinary extracts.
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PMID:Apparent heterogeneity of human megakaryocyte colony- and thrombopoiesis-stimulating factors: studies on urinary extracts from patients with aplastic anaemia and idiopathic thrombocytopenic purpura. 698 64


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