摘要:

AbstractErythrocyte lysate proteins prepared by five methods were chemically modified by methanethiolation with methyl methanethiolsulfonate (MMTS). This relatively newly studied agent delivers the small uncharged, non‐hydrogen‐bonding CH3S‐group to accessible ‐SH groups under mild conditions. Glycolytic and nonglycolytic red cell enzymes studied could be grouped into four categories based on degree of catalytic inhibition by MMTS, which ranged from 100% to nil. NADH (0.06 mM) protected against lactate dehydrogenase (LDH) inhibition nearly completely, and partial protection was evident at 1 μM concentrations. NAD was virtually ineffective at comparable molarities, and pyruvate failed to protect significantly. Glucose‐6‐phosphate dehydrogenase (G‐6‐PD) (but not 6‐PGD) was protected by NADP (but minimally if at all by NADPH) at low molarities. Substrate G‐6‐P failed to protect. Mg++alone protected enolase to a major degree against MMTS inhibition. Residual activities of several enzymes were rendered highly unstable to thermal stress, and in the case of selected enzymes, alteration of pH activity curves and catalytic activity at diminished substrate concentration was demonstrable. The highly specific modification of sulfhydryl groups of cysteinyl residues under mild conditions preserving other aspects of enzyme structure provides insights into the role of these most reactive of all amino side‐chains in the enzyme proteins derived fro

ISSN:0361-8609    

DOI:10.1002/ajh.2830110202

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractThe level of enzyme activity, the enzyme thermostability profile, and the isozyme electrophoretic pattern were determined in young and old erythrocytes from newborn infants and adults and in samples from adult individuals with increased reticulocyte counts. Cord blood samples had higher levels of enzymatic activity for 12 of the 14 enzymes measured, adenylate kinase and phos‐phoglucomutase being the exceptions. The largest differences in activity between newborns and adults were for glutamic oxaloacetic transaminase, hexo‐kinase, glucose 6‐phosphate dehydrogenase, and glutathione reductase, while glutamic oxaloacetic transaminase and pyruvate kinase showed the largest differences between young and old cells. The levels of activity of glutathione reductase, adenylate kinase, phosphoglucomutase, lactate dehydrogenase, phos‐phoglycerokinase, and glucose phosphate isomerase in cord blood samples suggest the regulation of expression of these enzymes is different in fetal erythrocytes than in erythrocytes from an adult. Differences in the thermostability profile of enzymes from cells from different sources and/or of different ages were noted for 5 of 9 enzymes. No unique electrophoretically identifiable fetal isozymes were observed, although differences in isozyme distribution and staining intensity associated with cell source and/or cell age were noted for many of the 23 enzymes examined. Many of these differences in enzyme characteristics have the potential to be confused with genetic alterations in enzyme structure and f

ISSN:0361-8609    

DOI:10.1002/ajh.2830110203

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractElectrophoretic analysis of a hemolysate from a young man undergoing a routine physical examination revealed an abnormal hemoglobin with a mobility similar to Hb S on cellulose acetate (pH, 8.4). This new variant, designated Hb Connecticut, was found in three generations of a family of Polish descent. Several individuals possessing the variant exhibited mild anemia. Structural analysis of the abnormal β‐chain indicated that the amino acid substitution was at position 21(B3), and involved the replacement of aspartic acid with glycine. Oxygen dissociation studies revealed low oxygen affinity. The alkaline Bohr effect and the degree of cooperativity were unchanged. Analysis of the crystal structure of the variant suggested that the low oxygen affinity was due to the possible disruption of salt bridges between aspartic acid 21(B3) and lysines 61(E5) and 65(E9), changes that could lead to steric interference in oxygen bindi

ISSN:0361-8609    

DOI:10.1002/ajh.2830110204

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractCirculating immune complexes (ICs), assayed by the L1210 enzyme‐linked immunoassay, were detected in 48% of patients with hemophilia A, 50% of patients with von Willebrand's disease, and in none of our patients with hemophilia B. Eighty‐five % of the hemophilia A and B patients had mild to moderate disease with only one patient demonstrating a circulating inhibitor. No correlation was found between IC levels and hepatitis B infection, SGOT, disease severity, total quantity of factor VIII or IX infused, time interval from last infusion, or rheumatoid factor positivity. Although the nature of the ICs is not known, the similarity of IC levels between hemophilia A and von‐Willebrand's disease is discussed with regard to antibodies generated to non‐procoagulant portions of the factor VIII m

ISSN:0361-8609    

DOI:10.1002/ajh.2830110205

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractTo identifiy causative factors responsible for the disseminated intravascular coagulation complicating peritoneovenous (LeVeen) shunts, the ascitic fluid from 12 patients with alcoholic liver disease or peritoneal malignancy was examined for its effects on human platelets. In all patients, concentrated ascitic fluid caused irreversible platelet aggregation. Properties of the aggregating factor suggested that it is collagen, and subsequently, the presence of collagen in ascitic fluid was confirmed.This finding, together with the known effects of collagen on platelets and contact clotting factors, would be sufficient to explain the development of disseminated intravascular coagulation following this procedure. Aspirin by inhibiting collagen‐induced aggregation may have a therapeutic role in the management of this proble

ISSN:0361-8609    

DOI:10.1002/ajh.2830110206

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractPlasma thromboxane A2, which is elevated during endotoxemia, has previously been shown to be a major factor contributing to the mortality and morbidity that occurs in endotoxin shock in the experimental animal. Using a minimal dose of Escherichia coli endotoxin (1 μg/ml), we have demonstrated that the preincubation of human platelets with endotoxin induces changes in platelet arachidonic acid release and the subsequent conversion of the released arachidonic acid to thromboxane B2, the stable end product of thromboxane A2. In paired experiments, in the presence of endotoxin, the addition of the aggregating agent thrombin (0.5 U/ml) caused human platelets to release 29.1 ± 3.4% of14C‐arachidonic acid from prelabeled platelet phospholipids. This value was significantly elevated (P<0.02) when compared with the release of14C‐arachidonic acid from platelets in the absence of endotoxin (21.9 ± 3.6%). Similarly, comparison of the results of the conversion of the released arachidonic acid to platelet thromboxane B2(TxB2) revealed that TxB2production was significantly increased (P<0.01) when human platelets were preincubated with endotoxin prior to the addition of thrombin (6.1 ± 0.6%) when compared with TxB2formation observed in the absence of endotoxin (3.4 ± 0.5%). The absolute amount of released arachidonic acid that was converted to TxB2in the presence of endotoxin (1.8 ± 0.3%) was also significantly higher (P<0.01) than the value observed in its absence (0.8 ± 0.2%). This study suggests that one of the tissue sources of the proaggregatory vasoconstrictor thromboxane A2during endotoxemia is th

ISSN:0361-8609    

DOI:10.1002/ajh.2830110207

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractA significant percentage of lymphoid cells isolated from three lymphoid neoplasms (nodular lymphoma mixed cell type, mycosis fungoides, and Sézary syndrome) had tetraploid or near‐tetraploid DNA content, as determined by flow cytometric analysis. In each case, a morphologically distinct population of cells with large, irregular nuclei was present, the percentage of which corresponded to the percentage of tetraploid cells. In a fourth case (diffuse “histiocytic” lymphoma), cells were sorted on the basis of DNA content from the G0‐G1compartment and compared morphologically to cells sorted from the S‐G2(M) compartment. The G0‐G1cells had clumped nuclear chromatin, lacked prominent nucleoli, and had a mean nuclear diameter of 10.18 ± 1.22 μm. The sorted cells with S‐G2(M) DNA content had one to several prominent nucleoli and less clumped nuclear chromatin, and they were significantly larger, with a mean nuclear diameter of 13.76 ± 1.59 μm (P<0.00001). These preliminary results suggest that, in lymphoid malignancies, certain morphologic characteristics of individual lymphoid cells are related to their DNA content (level of ploidy) and/or the stage of the cell within

ISSN:0361-8609    

DOI:10.1002/ajh.2830110208

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractIn order to obtain further knowledge of chronic neutropenia of childhood, we studied nine neutropenic infants six to ten months of age by in vitro techniques, including bone marrow culture, electron microscopy, and chemotaxis assay. Eight of the nine patients had a benign clinical course and the bone marrow aspirates showed a reduced number of segmented neutrophils. The ninth patient had a moderately severe course and the bone marrow showed maturation arrest at the promyelocyte stage. Bone marrow cultures demonstrated that the in vitro neutrophil colony formation and production of colony‐stimulating activity were normal in all of the eight patients studied. Neutrophils from one of the nine patients had ultrastructural abnormalities such as a decrease in number of primary and secondary granules and the presence of myelin figures in primary granules. Neutrophil chemotaxis was defective in three of the nine patients. All of the six patients in whom the neutrophil colony formation in agar, the ultrastructure of neutrophils, and neutrophil chemotaxis were normal recovered from the neutropenia between 11 and 30 months of age. These in vitro parameters appear to be useful for evaluating chronic neutropenia of childhoo

ISSN:0361-8609    

DOI:10.1002/ajh.2830110209

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractMethods for the standardization of several currently used platelet function tests (bleeding time, capillary fragility, platelet retention, platelet aggregation, platelet factor 3, and platelet volume profiles) are presented. Different variables that may interfere with the reproducibility of the results of each assay were identified and standardized. Using the standardized techniques, the range of normal values for each test was determined in a large population of normal volunteers and used to identify disease states by comparing patient results with those of the normal population. A format for presenting the entire profile of platelet function parameters is proposed.

ISSN:0361-8609    

DOI:10.1002/ajh.2830110210

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY

摘要:

AbstractA female with chronic myeloid leukemia (XX Ph 1 +) in blast crisis (localized to pleura and lymph nodes) was treated by polychemotherapy. After reversion to the chronic phase, an allogeneic bone marrow transplantation (BMT) was performed. Sixteen months after BMT, no sign of the disease was present (XY Ph 1 ‐

ISSN:0361-8609    

DOI:10.1002/ajh.2830110211

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1981

数据来源: WILEY