摘要:

AbstractA platelet kinetic study was performed in 34 patients (33 homosexuals and/or drug addicts) with (in most of them) severe and isolated thrombocytopenia and human immunodeficiency virus positive serological tests. The reported data indicate that the thrombocytopenia is due to an extracorpuscular hyperdestruction. The sequestration of the labeled platelets is exclusively splenic in most of the cases. Splenectomy was successful in the ten cases in which it was done; however, corticoids showed no or low effect, and Ig‐G infusion had only a temporary efficac

ISSN:0361-8609    

DOI:10.1002/ajh.2830260402

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe clinical, pigmentary, and ceroid storage manifestations of the Hermansky‐Pudlak syndrome (HPS) triad of albinism, hemorrhagic diathesis, and ceroid storage disease are variable. Therefore, a rapid and accurate method of diagnosing HPS is needed. Platelets of 66 albinos were examined by electron microscopy for the presence or absence of dense bodies. Results show that patients reexamined over a period of 1 year had consistent findings. Those lacking dense bodies (15) when first examined also lacked dense bodies when reexamined a year later, and they had evidence of ceroid storage. Those with dense bodies when first examined (8) also had dense bodies when reexamined, did not have evidence of storage disease, and had types of albinism other than HPS. Of 20 propositi lacking dense bodies, all 32 albino relatives also lacked dense bodies, while 6 albino relatives of 6 propositi with dense bodies also had dense bodies in their platelets. The evidence supports the concept that HPS is a distinct genetic and biochemical disease in which the components of the triad are the result of a single genetic defect, either a point mutation or a small deletion. Comparison of whole mount preparations with thin section preparations of 13 albinos shows that whole mount preparations are an accurate and rapid method for diagnosing HPS. The most consistent diagnostic feature of HPS is lack of platelet dense bodie

ISSN:0361-8609    

DOI:10.1002/ajh.2830260403

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

年代:1987

数据来源: WILEY

摘要:

AbstractAn evaluation of a new Enzyme Linked Immunosorbent Assay (ELISA) factor VII:Ag assay in factor VII congenital deficiencies was carried out. This assay was compared to factor VII:C assay and the Inhibitor Neutralization Assay (INA) for factor VII:Ag, both in normals and F VII‐deficient patients. The correlations between ELISA F VII:Ag and VII:C, as well as the one between INA F VII:Ag and VII:C, were good (r= .86 and .81, respectively). The correlation between the two immunologic methods of assay in normals was fairly good (r= 0.73,P<0.001); whereas in mild F VII deficiencies (heterozygotes for F VII deficiencies), the two methods correlated very well (r= .96). In the severe deficiencies, ELISA F VII:Ag assay allowed the evaluation of F VII protein levels below 1 u/dl.The sensitivities of the INA and ELISA assays were evaluated by creating artificially prepared plasmas containing serial amounts of purified F VII: INA was unable to pick up F VII protein levels lower than 75 ng/ml, whereas the ELISA assay could detect up to 5 ng of F VI

ISSN:0361-8609    

DOI:10.1002/ajh.2830260404

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

年代:1987

数据来源: WILEY

摘要:

AbstractFour factor XI (F XI)‐deficient patients are described, all of whom formed circulating anticoagulants against F X1. In the three most severely affected patients (F XI 0%–6% activity), the anticoagulant appeared to have been stimulated by plasma infusion. However, in the milder case (25% F XI activity), no infusion had been documented. The findings in these cases emphasize the diversity of F XI inhibitors in congenitally deficient patients. Awareness of the potential development of these inhibitors will be helpful in both daily management and perioperative care of such patie

ISSN:0361-8609    

DOI:10.1002/ajh.2830260405

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

年代:1987

数据来源: WILEY

摘要:

AbstractHemoglobin H (HbH) disease is most often due to deletion of three of the four α‐globin genes (genotype ‐ ‐ ‐ ‐/‐α). In black subjects although the ‐α/chromosome is common, the ‐ ‐/haplotype is very rare and few examples of HbH disease have been detected. We have studied three black siblings with HbH by restriction endonuclease mapping of the α‐like gene complex (5′‐ζ‐Ψζ‐Ψα2‐Ψα1‐α2‐α1‐3′) using ζ‐ and α‐ specific probes. The presence of size differences in the previously described hypervariable region between the ζ and ζΨ genes results in a restriction fragment length polymorphism which permitted the detection of single α genes on both number 16 chromosomes in these subjects. Quantitative DNA hybridization by a slot‐blot technique confirmed that their genomes contained two α‐globin genes. The results establish that in these black subjects HbH disease i

ISSN:0361-8609    

DOI:10.1002/ajh.2830260406

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

年代:1987

数据来源: WILEY

摘要:

AbstractPlatelet crossmatching assays have been used to predict the outcome of platelet transfusions in alloimmunized patients by detecting antibodies against platelets. The transfusion failure of HLA‐matched platelets predicted by platelet crossmatching may be related to HLA antibodies undetected by lymphocytotoxicity but detected by platelet immunoglobulin‐binding assays or platelet‐specific antibodies (both antibodies defined here as platelet‐reactive antibodies). To differentiate platelet‐reactive antibodies from lymphocytotoxic HLA antibodies, we used HLA characterized lymphocytes in parallel with platelets from individuals to form separate frozen panels. Sera from 10 allosensitized patients were studied in the lymphocyte panel by lymphocytotoxicity and in the platelet panel by enzyme‐linked immunoassay (ELISA). By comparing pattern and percent wells reacting in each panel, lymphocytotoxic HLA antibodies and antibodies reactive with platelets in ELISA were detected separately. In all 10 allosensitized patients, platelet‐associated antibodies were present and 7 had additional lymphocytotoxic HLA antibodies. Using this double parallel panel technique, we found platelet‐reactive antibodies important in platelet alloimmunization, unrecognized by lymphocytotoxicity. These data indicate platelet‐crossmatching be solely used in the selection of platelets for allose

ISSN:0361-8609    

DOI:10.1002/ajh.2830260407

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

年代:1987

数据来源: WILEY

摘要:

AbstractThe half‐saturation constant (K0.5s) phosphoenolpyruvate (PEP) for red cell pyruvate kinase (PK) with co‐factors UDP and GDP is less than one‐half that with ADP with or without additions of the allosteric modifier, fructose‐1, 6‐dephosphate (F‐1, 6‐P2) to the assay. The Vmax is markedly greater with ADP than with UDP or GDP, but with (PEP) at 0.5 mM, activity with all co‐factors is about equal and at lower concentrations greater with UDP and GDP. With high K0.5s(PEP) mutant enzymes, and at the usual test concentration (1mM) for PEP when nucleotide specificity is assessed, the abnormally low saturation of variant enzymes may result in higher activity with UDP and GDP than with ADP–the opposite of the “normal situation.” The apparent aberration in nucleotide specificity may thus be illusory and secondary to the abnormal K0.5s(PEP) of the mutant. Example data are recorded. Variations in K0.5s(PEP) may also be introduced during enzyme preparation for assay, particularly when partial pur

ISSN:0361-8609    

DOI:10.1002/ajh.2830260408

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

年代:1987

数据来源: WILEY

摘要:

AbstractIn this article, we have reviewed studies showing that altered expression of regulatory genes results in activation of embryonic genes in human leukemia cells. These data have led to important new insights as to how mutations in regulatory genes can lead to disease states in man.

ISSN:0361-8609    

DOI:10.1002/ajh.2830260409

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

年代:1987

数据来源: WILEY

ISSN:0361-8609    

DOI:10.1002/ajh.2830260401

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

年代:1987

数据来源: WILEY