Prothrombin Houston: a dysprothrombin identifiable by crossed immunoelectrofocusing and abnormal Echis carinatus venom activation.

Abstract:

:A 72-yr-old male with a lifelong history of easy bruisability and posttraumatic bleeding had a prolonged prothrombin time and activated partial thromboplastin time. His plasma Stypven, Taipan, and Echis carinatus venom clotting times were prolonged. The presence of a dysprothrombin was confirmed by the discrepancy between plasma prothrombin coagulant activity and prothrombin antigen levels. His plasma prothrombin was capable of being completely absorbed onto and then eluted from barium sulfate. Crossed immunoelectrophoresis of his plasma prothrombin, and normal plasma prothrombin, into agarose containing rabbit anti-human factor II antibody were similar. Crossed immunoelectrofocusing, a procedure combining isoelectric focusing in disc gels with electroimmunoassay in the second dimension, demonstrated that the patient's prothrombin antigen was more basic than normal. The eluate from barium sulfate absorbtion of patient plasma, when reacted with Echis carinatus venom (which directly cleaves prothrombin to thrombin) clotted purified fibrinogen at a rate slower than normal plasma eluate. SDS-slab gel electrophoresis revealed that the prothrombin present in the patient's eluate was cleaved by Echis carinatus venom. These studies suggest that the coagulopathy of prothrombin Houston results from the generation of a dysfunctional thrombin.

journal_name

Blood

journal_title

Blood

authors

Weinger RS,Rudy C,Moake JL,Olson JD,Cimo PL

subject

Has Abstract

pub_date

1980-05-01 00:00:00

pages

811-6

issue

5

eissn

0006-4971

issn

1528-0020

journal_volume

55

pub_type

杂志文章

相关文献

BLOOD文献大全
  • Ferroportin deficiency in erythroid cells causes serum iron deficiency and promotes hemolysis due to oxidative stress.

    abstract::Ferroportin (FPN), the only known vertebrate iron exporter, transports iron from intestinal, splenic, and hepatic cells into the blood to provide iron to other tissues and cells in vivo. Most of the circulating iron is consumed by erythroid cells to synthesize hemoglobin. Here we found that erythroid cells not only co...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2018-04-842997

    authors: Zhang DL,Ghosh MC,Ollivierre H,Li Y,Rouault TA

    更新日期:2018-11-08 00:00:00

  • Persistent activation of the tumor necrosis factor system in a subgroup of patients with common variable immunodeficiency--possible immunologic and clinical consequences.

    abstract::In patients with common variable immunodeficiency (CVI), we have previously defined a subgroup of patients (CVIHyper) characterized by decreased numbers of CD4+ lymphocytes in peripheral blood, splenomegaly, and persistent immune activation in vivo, particularly of monocytes/macrophages. To further characterize this h...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Aukrust P,Lien E,Kristoffersen AK,Müller F,Haug CJ,Espevik T,Frøland SS

    更新日期:1996-01-15 00:00:00

  • p53 and N-ras mutations in two new leukemia cell lines established from a patient with multilineage CD7-positive acute leukemia.

    abstract::Two new myeloid cell lines (K051 and K052) were established from a patient with multilineage CD7-positive acute leukemia. The K051 and K052 were established from the patient's bone marrow cells at diagnosis and at relapse, respectively. The K051 cell expressed myeloid-associated antigens (CD13 and CD33), a platelet-as...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Abo J,Inokuchi K,Dan K,Nomura T

    更新日期:1993-11-01 00:00:00

  • Results of high-dose therapy and autologous bone marrow/stem cell transplantation during remission in poor-risk intermediate- and high-grade lymphoma: international index high and high-intermediate risk group.

    abstract::We have conducted a pilot study to investigate the role of high-dose therapy and autologous bone marrow/stem cell transplantation (ASCT) during first complete or partial remission in 52 patients with poor-risk aggressive lymphoma. There were 42 patients with intermediate-grade or immunoblastic lymphoma who were consid...

    journal_title:Blood

    pub_type: 临床试验,杂志文章

    doi:

    authors: Nademanee A,Molina A,O'Donnell MR,Dagis A,Snyder DS,Parker P,Stein A,Smith E,Planás I,Kashyap A,Spielberger R,Fung H,Wong KK,Somlo G,Margolin K,Chow W,Sniecinski I,Vora N,Blume KG,Niland J,Forman SJ

    更新日期:1997-11-15 00:00:00

  • Fish oil: a potent inhibitor of platelet adhesiveness.

    abstract::The effect of fish oil administration on platelet function was studied in eight normal individuals, four men and four women, who received fish oil equivalent to 6 g eicosapentaenoic acid per day for a period of 25 days. Platelet aggregation, platelet adhesion, phospholipid and fatty acid distribution were measured at ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Li XL,Steiner M

    更新日期:1990-09-01 00:00:00

  • Phosphotyrosine profiling identifies the KG-1 cell line as a model for the study of FGFR1 fusions in acute myeloid leukemia.

    abstract::The 8p11 myeloproliferative syndrome (EMS) is associated with translocations that disrupt the FGFR1 gene. To date, 8 fusion partners of FGFR1 have been identified. However, no primary leukemia cell lines were identified that contain any of these fusions. Here, we screened more than 40 acute myeloid leukemia cell lines...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2006-06-026666

    authors: Gu TL,Goss VL,Reeves C,Popova L,Nardone J,Macneill J,Walters DK,Wang Y,Rush J,Comb MJ,Druker BJ,Polakiewicz RD

    更新日期:2006-12-15 00:00:00

  • Escape from suppression: tumor-specific effector cells outcompete regulatory T cells following stem-cell transplantation.

    abstract::Immune reconstitution of autologous hematopoietic stem-cell transplant recipients with the progeny of mature T cells in the graft leads to profound changes in the emerging functional T-cell repertoire. In the steady state, the host is frequently tolerant to tumor antigens, reflecting dominant suppression of naive and ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2007-06-096586

    authors: Mirmonsef P,Tan G,Zhou G,Morino T,Noonan K,Borrello I,Levitsky HI

    更新日期:2008-02-15 00:00:00

  • Chromosome translocations involving band 7q35 or 7p15 in childhood T-cell leukemia/lymphoma.

    abstract::In a chromosome study in childhood T-cell leukemia/lymphoma, we found t(7;11)(q35;p13) in 2 patients, t(7;14) (q35;q11) in one patient, and t(7;14)(p15;q32) in 1 patient. Southern blotting and in situ chromosomal hybridization studies in one patient with the t(7;11) demonstrated that both alleles of the T-cell antigen...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Kaneko Y,Maseki N,Homma C,Sakurai M,Mizutani S,Takeda T,Shikano T,Fujimoto T,Yaoi K,Shimokawa T

    更新日期:1988-08-01 00:00:00

  • Baboon envelope pseudotyped LVs outperform VSV-G-LVs for gene transfer into early-cytokine-stimulated and resting HSCs.

    abstract::Hematopoietic stem cell (HSC)-based gene therapy holds promise for the cure of many diseases. The field is now moving toward the use of lentiviral vectors (LVs) as evidenced by 4 successful clinical trials. These trials used vesicular-stomatitis-virus-G protein (VSV-G)-LVs at high doses combined with strong cytokine-c...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2014-02-558163

    authors: Girard-Gagnepain A,Amirache F,Costa C,Lévy C,Frecha C,Fusil F,Nègre D,Lavillette D,Cosset FL,Verhoeyen E

    更新日期:2014-08-21 00:00:00

  • Clonal B cells in patients with hepatitis C virus-associated mixed cryoglobulinemia contain an expanded anergic CD21low B-cell subset.

    abstract::Hepatitis C virus (HCV) is associated with the B-cell lymphoproliferative disorders mixed cryoglobulinemia (MC) and non-Hodgkin lymphoma. We have previously reported that HCV(+)MC(+) patients have clonal expansions of hypermutated, rheumatoid factor-bearing marginal zone-like IgM(+)CD27(+) peripheral B cells using the...

    journal_title:Blood

    pub_type: 临床试验,杂志文章

    doi:10.1182/blood-2010-10-312942

    authors: Charles ED,Brunetti C,Marukian S,Ritola KD,Talal AH,Marks K,Jacobson IM,Rice CM,Dustin LB

    更新日期:2011-05-19 00:00:00

  • Efficacy of venetoclax in relapsed chronic lymphocytic leukemia is influenced by disease and response variables.

    abstract::To define the efficacy of venetoclax with extended follow-up and identify clinical or biological treatment effect modifiers, updated data for previously treated patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) enrolled in 4 early-phase trials were pooled. Rates of response, complete...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood.2018882555

    authors: Roberts AW,Ma S,Kipps TJ,Coutre SE,Davids MS,Eichhorst B,Hallek M,Byrd JC,Humphrey K,Zhou L,Chyla B,Nielsen J,Potluri J,Kim SY,Verdugo M,Stilgenbauer S,Wierda WG,Seymour JF

    更新日期:2019-07-11 00:00:00

  • A novel negative regulatory function of the phosphoprotein associated with glycosphingolipid-enriched microdomains: blocking Ras activation.

    abstract::In primary human T cells, anergy induction results in enhanced p59Fyn activity. Because Fyn is the kinase primarily responsible for the phosphorylation of PAG (the phosphoprotein associated with glycosphingolipid-enriched microdomains), which negatively regulates Src-kinase activity by recruiting Csk (the C-terminal S...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2006-07-038752

    authors: Smida M,Posevitz-Fejfar A,Horejsi V,Schraven B,Lindquist JA

    更新日期:2007-07-15 00:00:00

  • The Inab phenotype: characterization of the membrane protein and complement regulatory defect.

    abstract::Recent demonstration that Cromer-related human blood group antigens reside on decay-accelerating factor (DAF) has led to identification of an apparent null phenotype (Inab) for erythrocyte DAF. This study examined expression of other phosphatidylinositol (PI)-anchored proteins by Inab erythrocytes and showed that the ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Telen MJ,Green AM

    更新日期:1989-07-01 00:00:00

  • Anti-factor VIII antibodies of hemophiliac patients are frequently directed towards nonfunctional determinants and do not exhibit isotypic restriction.

    abstract::A significant proportion of hemophilia A patients receiving transfusions of factor VIII (FVIII) develop a specific antibody response towards FVIII. These antibodies are usually detected by assays in which they inhibit the function of the molecule, such as the Bethesda clotting test. We have prepared anti-FVIII antibod...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Gilles JG,Arnout J,Vermylen J,Saint-Remy JM

    更新日期:1993-10-15 00:00:00

  • Sustained phenotypic correction of canine hemophilia A using an adeno-associated viral vector.

    abstract::Gene therapy for hemophilia A requires efficient delivery of the factor VIII gene and sustained protein expression at circulating levels of at least 1% to 2% of normal. Adeno-associated viral type 2 (AAV2) vectors have a number of advantages over other viral vectors, including an excellent safety profile and persisten...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2003-01-0292

    authors: Scallan CD,Lillicrap D,Jiang H,Qian X,Patarroyo-White SL,Parker AE,Liu T,Vargas J,Nagy D,Powell SK,Wright JF,Turner PV,Tinlin SJ,Webster SE,McClelland A,Couto LB

    更新日期:2003-09-15 00:00:00

  • Protein A is the von Willebrand factor binding protein on Staphylococcus aureus.

    abstract::Endovascular infection is a highly critical complication of invasive Staphylococcus aureus disease. For colonization, staphylococci must first adhere to adhesive endovascular foci. Von Willebrand factor (vWF) is a large, multimeric glycoprotein mediating platelet adhesion at sites of endothelial damage. Earlier it was...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Hartleib J,Köhler N,Dickinson RB,Chhatwal GS,Sixma JJ,Hartford OM,Foster TJ,Peters G,Kehrel BE,Herrmann M

    更新日期:2000-09-15 00:00:00

  • SLFN11 promotes stalled fork degradation that underlies the phenotype in Fanconi anemia cells.

    abstract::Fanconi anemia (FA) is a hereditary disorder caused by mutations in any 1 of 22 FA genes. The disease is characterized by hypersensitivity to interstrand crosslink (ICL) inducers such as mitomycin C (MMC). In addition to promoting ICL repair, FA proteins such as RAD51, BRCA2, or FANCD2 protect stalled replication fork...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood.2019003782

    authors: Okamoto Y,Abe M,Mu A,Tempaku Y,Rogers CB,Mochizuki AL,Katsuki Y,Kanemaki MT,Takaori-Kondo A,Sobeck AT,Bielinsky AK,Takata M

    更新日期:2020-07-31 00:00:00

  • Acute onset hemoglobinemia and/or hemoglobinuria and sequelae following Rh(o)(D) immune globulin intravenous administration in immune thrombocytopenic purpura patients.

    abstract::Rh(o)(D) immune globulin intravenous (anti-D IGIV) was licensed by the United States Food and Drug Administration (FDA) in March 1995 to treat patients with immune thrombocytopenic purpura (ITP). Anti-D IGIV induces extravascular hemolysis, an expected adverse reaction that is consistent with the presumed mechanism of...

    journal_title:Blood

    pub_type: 杂志文章,评审

    doi:

    authors: Gaines AR

    更新日期:2000-04-15 00:00:00

  • Characterization and use of an antibody detecting the CBFbeta-SMMHC fusion protein in inv(16)/t(16;16)-associated acute myeloid leukemias.

    abstract::The inv(16)(p13q22) and t(16;16)(p13;q22) cytogenetic abnormalities occur commonly in acute myeloid leukemia (AML), typically associated with French-American-British (FAB) AML-M4Eo subtype. Reverse transcriptase-polymerase chain reaction (RT-PCR) techniques have been recently developed to detect the presence of severa...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Viswanatha DS,Chen I,Liu PP,Slovak ML,Rankin C,Head DR,Willman CL

    更新日期:1998-03-15 00:00:00

  • miR-155 and its star-form partner miR-155* cooperatively regulate type I interferon production by human plasmacytoid dendritic cells.

    abstract::The recent discovery of microRNAs (miRNAs) has revealed a new layer of gene expression regulation, affecting the immune system. Here, we identify their roles in regulating human plasmacytoid dendritic cell (PDC) activation. miRNA profiling showed the significantly differential expression of 19 miRNAs in PDCs after Tol...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2010-04-280156

    authors: Zhou H,Huang X,Cui H,Luo X,Tang Y,Chen S,Wu L,Shen N

    更新日期:2010-12-23 00:00:00

  • p15ink4B and p16ink4 gene inactivation in acute lymphocytic leukemia.

    abstract::Malignant cells from 52 children with acute lymphocytic leukemia (ALL) were investigated for inactivation of the p15ink4B and p16ink4 genes and other genetic alterations on chromosome 9p21. Homozygous deletions of the p15ink4B and/or the p16ink4 genes were detected in 16 cases and a further 9 cases showed evidence of ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Rasool O,Heyman M,Brandter LB,Liu Y,Grandér D,Söderhäll S,Einhorn S

    更新日期:1995-06-15 00:00:00

  • A novel form of immune signaling revealed by transmission of the inflammatory mediator serotonin between dendritic cells and T cells.

    abstract::Adaptive immunity is triggered at the immune synapse, where peptide-major histocompatibility complexes and costimulatory molecules expressed by dendritic cells (DCs) are physically presented to T cells. Here we describe transmission of the inflammatory monoamine serotonin (5-hydroxytryptamine [5-HT]) between these cel...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2005-07-2903

    authors: O'Connell PJ,Wang X,Leon-Ponte M,Griffiths C,Pingle SC,Ahern GP

    更新日期:2006-02-01 00:00:00

  • A novel role for IL-22R1 as a driver of inflammation.

    abstract::The interleukin (IL)-22R1 chain of the heterodimeric IL-22 receptor is not expressed on normal leukocytes, but this receptor is expressed on T cells from anaplastic lymphoma kinase-positive (ALK(+)) anaplastic large cell lymphoma (ALCL) patients. To investigate the consequences of aberrant expression of this receptor ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2010-05-285908

    authors: Savan R,McFarland AP,Reynolds DA,Feigenbaum L,Ramakrishnan K,Karwan M,Shirota H,Klinman DM,Dunleavy K,Pittaluga S,Anderson SK,Donnelly RP,Wilson WH,Young HA

    更新日期:2011-01-13 00:00:00

  • Steady-state generation of mucosal IgA+ plasmablasts is not abrogated by B-cell depletion therapy with rituximab.

    abstract::The anti-CD20 antibody rituximab depletes human B cells from peripheral blood, but it remains controversial to what extent tissue-resident B cells are affected. In representative patients with rheumatoid arthritis, we here demonstrate that recently activated presumably short-lived plasmablasts expressing HLA-DR(high) ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2010-01-266536

    authors: Mei HE,Frölich D,Giesecke C,Loddenkemper C,Reiter K,Schmidt S,Feist E,Daridon C,Tony HP,Radbruch A,Dörner T

    更新日期:2010-12-09 00:00:00

  • Mechanism for fetal hemoglobin induction by histone deacetylase inhibitors involves gamma-globin activation by CREB1 and ATF-2.

    abstract::The histone deacetylase inhibitors (HDA-CIs) butyrate and trichostatin A activate gamma-globin expression via a p38 mitogen-activating protein kinase (MAPK)-dependent mechanism. We hypothesized that down-stream effectors of p38 MAPK, namely activating transcription factor-2 (ATF-2) and cyclic AMP response element (CRE...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2006-01-023713

    authors: Sangerman J,Lee MS,Yao X,Oteng E,Hsiao CH,Li W,Zein S,Ofori-Acquah SF,Pace BS

    更新日期:2006-11-15 00:00:00

  • Functional characterization of the human dendritic cell immunodeficiency associated with the IRF8(K108E) mutation.

    abstract::We have previously reported on a unique patient in whom homozygosity for a mutation at IRF8 (IRF8(K108E)) causes a severe immunodeficiency. Laboratory evaluation revealed a highly unusual myeloid compartment, remarkable for the complete absence of CD141 and CD161 monocytes, absence of CD11c1 conventional dendritic cel...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2014-04-570879

    authors: Salem S,Langlais D,Lefebvre F,Bourque G,Bigley V,Haniffa M,Casanova JL,Burk D,Berghuis A,Butler KM,Leahy TR,Hambleton S,Gros P

    更新日期:2014-09-18 00:00:00

  • Erythropoietin receptor haploinsufficiency and in vivo interplay with granulocyte-macrophage colony-stimulating factor and interleukin 3.

    abstract::Erythropoietin (EPO) and its receptor (EPOR) are critical for definitive erythropoiesis, as mice lacking either gene product die during embryogenesis with severe anemia. Here we demonstrate that mice expressing just one functional allele of the EpoR have lower hematocrits and die more frequently than do wild-type litt...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood.v99.7.2603

    authors: Jegalian AG,Acurio A,Dranoff G,Wu H

    更新日期:2002-04-01 00:00:00

  • An autoantibody directed against human thrombin anion-binding exosite in a patient with arterial thrombosis: effects on platelets, endothelial cells, and protein C activation.

    abstract::An autoantibody, developed by a patient with severe and recurrent arterial thrombosis, was characterized to be directed against the anion-binding exosite of thrombin, and inhibited all thrombin interactions requiring this secondary binding site without interfering with the catalytic site. The effect of the antibody wa...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Arnaud E,Lafay M,Gaussem P,Picard V,Jandrot-Perrus M,Aiach M,Rendu F

    更新日期:1994-09-15 00:00:00

  • Expression of Pitx2 in stromal cells is required for normal hematopoiesis.

    abstract::Although the expression of Pitx2, a bicoid family homeodomain transcription factor, is highly regulated during hematopoiesis, its function during this process was not documented; we thus studied hematopoiesis in Pitx2-null mice. We found that Pitx2(-/-) embryos display hypoplastic livers with reduced numbers of hemato...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2005-02-0529

    authors: Kieusseian A,Chagraoui J,Kerdudo C,Mangeot PE,Gage PJ,Navarro N,Izac B,Uzan G,Forget BG,Dubart-Kupperschmitt A

    更新日期:2006-01-15 00:00:00

  • Cyclophosphamide enhances immunity by modulating the balance of dendritic cell subsets in lymphoid organs.

    abstract::Cyclophosphamide (CTX), a commonly used chemotherapeutic agent can enhance immune responses. The ability of CTX to promote the proliferation of effector T cells and abrogate the function of regulatory T cells (Tregs) has been described. In this study, we examined the effects of CTX treatment on dendritic cell (DC) sub...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2009-11-251231

    authors: Nakahara T,Uchi H,Lesokhin AM,Avogadri F,Rizzuto GA,Hirschhorn-Cymerman D,Panageas KS,Merghoub T,Wolchok JD,Houghton AN

    更新日期:2010-06-03 00:00:00