Acquired von Willebrand's disease in association with Wilm's tumor: regression following treatment.

Abstract:

:A 9-yr-old female presented with a Wilm's tumor and a coagulopathy consistent with von Willebrand's disease. Factor VIII procoagulant activity (VIII C), factor VIII related antigen (VIIIR:Ag), and von Willebrand factor activity (VIII:vWf) were decreased. There was no evidence for a circulating inhibitor of the factor VIII molecular complex. von Willebrand's antigen II (vW AgII), which is deficient in hereditary von Willebrand's disease, was decreased below detectable levels in this patient. The coagulation studies, VIIIR:Ag, and vW AgII levels returned to normal following therapy of the Wilm's tumor. Wilm's tumor must be included as one of the malignancies associated with acquired von Willebrand's disease. Immunofluorescent studies of the tumor specimen showed normal endothelial staining of VIIIR:Ag by semiquantitative techniques and a lack of specific tumor adsorption of VIIIR:Ag The presence of normal amounts of tissue VIIIR:Ag has not previously been demonstrated in acquired von Willebrand's disease. Since we failed to demonstrate an inhibitor in the plasma in this patient, the etiology of the acquired von Willebrand's disease in this patient appears to differ from other cases of acquired von Willebrand's disease. The finding that vW AgII is decreased in this patient, similar to that reported in hereditary von Willebrand's disease, supports the close association of vW AgII to VIIIR:Ag, even though they are immunologically and biochemically distinct.

journal_name

Blood

journal_title

Blood

authors

Scott JP,Montgomery RR,Tubergen DG,Hays T

subject

Has Abstract

pub_date

1981-10-01 00:00:00

pages

665-9

issue

4

eissn

0006-4971

issn

1528-0020

journal_volume

58

pub_type

杂志文章

相关文献

BLOOD文献大全
  • Differential surrogate light chain expression governs B-cell differentiation.

    abstract::Surrogate light chain expression during B lineage differentiation was examined by using indicator fluorochrome-filled liposomes in an enhanced immunofluorescence assay. Pro-B cells bearing surrogate light chain components were found in mice, but not in humans. A limited subpopulation of relatively large pre-B cells in...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood.v99.7.2459

    authors: Wang YH,Stephan RP,Scheffold A,Kunkel D,Karasuyama H,Radbruch A,Cooper MD

    更新日期:2002-04-01 00:00:00

  • The Src kinase Lyn is required for CCR5 signaling in response to MIP-1beta and R5 HIV-1 gp120 in human macrophages.

    abstract::CCR5 is a receptor for several beta chemokines and the entry coreceptor used by macrophage-tropic (R5) strains of HIV-1. In addition to supporting viral entry, CCR5 ligation by the HIV-1 envelope glycoprotein 120 (gp120) can activate intracellular signals in macrophages and trigger inflammatory mediator release. Using...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2005-12-012815

    authors: Tomkowicz B,Lee C,Ravyn V,Cheung R,Ptasznik A,Collman RG

    更新日期:2006-08-15 00:00:00

  • VavP-Bcl2 transgenic mice develop follicular lymphoma preceded by germinal center hyperplasia.

    abstract::In human follicular lymphoma the t(14; 18) chromosome translocation activates the antiapoptotic oncogene Bcl2 by linking it to the immunoglobulin heavy chain (IGH) locus. Transgenic mice expressing Bcl2 controlled by an Igh enhancer (E mu) do not develop follicular lymphoma, although they do have an increased incidenc...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2003-07-2469

    authors: Egle A,Harris AW,Bath ML,O'Reilly L,Cory S

    更新日期:2004-03-15 00:00:00

  • CCR7-mediated physiological lymphocyte homing involves activation of a tyrosine kinase pathway.

    abstract::Homing of blood-borne lymphocytes to peripheral lymph nodes (PLNs) is a multistep process dependent on the sequential engagement of L-selectin, which mediates lymphocyte rolling along the luminal surface of high endothelial venules (HEVs), followed by activation of lymphocyte integrins and transmigration through HEVs....

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2002-03-0841

    authors: Stein JV,Soriano SF,M'rini C,Nombela-Arrieta C,de Buitrago GG,Rodríguez-Frade JM,Mellado M,Girard JP,Martínez-A C

    更新日期:2003-01-01 00:00:00

  • Membrane translocation of 15-lipoxygenase in hematopoietic cells is calcium-dependent and activates the oxygenase activity of the enzyme.

    abstract::Mammalian 15-lipoxygenases, which have been implicated in the differentiation of hematopoietic cells are commonly regarded as cytosolic enzymes. Studying the interaction of the purified rabbit reticulocyte 15-lipoxygenase with various types of biomembranes, we found that the enzyme binds to biomembranes when calcium i...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Brinckmann R,Schnurr K,Heydeck D,Rosenbach T,Kolde G,Kühn H

    更新日期:1998-01-01 00:00:00

  • Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the International Prognostic Index.

    abstract::Nasal natural killer (NK) cell lymphoma is rare, so that its optimal therapy, long-term outcome, and prognostic factors are unclear. Data on 52 men and 15 women with well-characterized nasal NK cell lymphomas were analyzed retrospectively to define the impact of primary therapy on remission and long-term outcome and t...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2003-05-1401

    authors: Chim CS,Ma SY,Au WY,Choy C,Lie AK,Liang R,Yau CC,Kwong YL

    更新日期:2004-01-01 00:00:00

  • Assembly of a fibronectin matrix by adherent platelets stimulated by lysophosphatidic acid and other agonists.

    abstract::Lysophosphatidic acid (LPA) and sphingosine-1-phosphate (S1P) are agonists of the endothelial differentiation gene (Edg) family of G-protein-coupled receptors. LPA and S1P are generated by platelet activation during blood coagulation. Both lipids induce assembly of exogenous fibronectin (FN) by fibroblasts. This study...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood.v98.1.117

    authors: Olorundare OE,Peyruchaud O,Albrecht RM,Mosher DF

    更新日期:2001-07-01 00:00:00

  • p55 and p75 tumor necrosis factor receptors in patients with chronic lymphocytic leukemia.

    abstract::We studied the expression of the two tumor necrosis factor (TNF) receptors, p55 and p75, on B cells from patients with chronic lymphocytic leukemia (CLL), and the presence of soluble TNF receptors in serum. Expression of membrane-associated receptors was quantified by double labeling of peripheral blood mononuclear ce...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Waage A,Liabakk N,Lien E,Lamvik J,Espevik T

    更新日期:1992-11-15 00:00:00

  • A case of paroxysmal nocturnal hemoglobinuria caused by a germline mutation and a somatic mutation in PIGT.

    abstract::To ascertain the genetic basis of a paroxysmal nocturnal hemoglobinuria (PNH) case without somatic mutations in PIGA, we performed deep next-generation sequencing on all exons of known genes of the glycosylphosphatidylinositol (GPI) anchor synthesis pathway. We identified a heterozygous germline splice site mutation i...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2013-01-481499

    authors: Krawitz PM,Höchsmann B,Murakami Y,Teubner B,Krüger U,Klopocki E,Neitzel H,Hoellein A,Schneider C,Parkhomchuk D,Hecht J,Robinson PN,Mundlos S,Kinoshita T,Schrezenmeier H

    更新日期:2013-08-15 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

  • Rare RHCE phenotypes in black individuals of Afro-Caribbean origin: identification and transfusion safety.

    abstract::The molecular backgrounds of variants encountered in Afro-Caribbean black individuals and associated with the production of clinically significant antibodies against high-incidence antigens (anti-RH18, anti-RH34) and against Rhe epitopes were determined. We showed that RH:-18 phenotypes are produced by 3 distinct RHCE...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2002-01-0229

    authors: Noizat-Pirenne F,Lee K,Pennec PY,Simon P,Kazup P,Bachir D,Rouzaud AM,Roussel M,Juszczak G,Ménanteau C,Rouger P,Kotb R,Cartron JP,Ansart-Pirenne H

    更新日期:2002-12-01 00:00:00

  • Characterization of human platelet glycoprotein antigens giving rise to individual immunoprecipitates in crossed-immunoelectrophoresis.

    abstract::Washed human platelets were labeled with 125I by the lactoperoxidase-catalyzed method and solubilized in 1% Triton X-100. The soluble proteins were analyzed by crossed-immunoelectrophoresis in 1% agarose, employing a rabbit antibody raised against whole human platelets. Analysis of autoradiograms developed from dried ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Kunicki TJ,Nurden AT,Pidard D,Russell NR,Caen JP

    更新日期:1981-12-01 00:00:00

  • High-dose cyclophosphamide, carmustine, and etoposide followed by autologous peripheral stem cell transplantation for patients with relapsed Hodgkin's disease.

    abstract::Between February 1986 and March 1990, 56 patients with relapsed Hodgkin's disease treated with high-dose cyclophosphamide, carmustine, and etoposide (CBV) received an autologous peripheral stem cell transplantation (PSCT) rather than an autologous bone marrow transplantation (ABMT) because each patient had a marrow ab...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Kessinger A,Bierman PJ,Vose JM,Armitage JO

    更新日期:1991-06-01 00:00:00

  • Tumor necrosis factor constellation polymorphism and clozapine-induced agranulocytosis in two different ethnic groups.

    abstract::Genes of the major histocompatibility complex (MHC) are associated with susceptibility to different immune and nonimmune mediated diseases. We had reported that the drug adverse reaction, clozapine-induced agranulocytosis (CA), is associated with different HLA types and HSP70 variants in Ashkenazi Jewish and non-Jewis...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Turbay D,Lieberman J,Alper CA,Delgado JC,Corzo D,Yunis JJ,Yunis EJ

    更新日期:1997-06-01 00:00:00

  • Platelet protein disulfide isomerase is required for thrombus formation but not for hemostasis in mice.

    abstract::Protein disulfide isomerase (PDI) derived from intravascular cells is required for thrombus formation. However, it remains unclear whether platelet PDI contributes to the process. Using platelet-specific PDI-deficient mice, we demonstrate that PDI-null platelets have defects in aggregation and adenosine triphosphate s...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2013-03-492504

    authors: Kim K,Hahm E,Li J,Holbrook LM,Sasikumar P,Stanley RG,Ushio-Fukai M,Gibbins JM,Cho J

    更新日期:2013-08-08 00:00:00

  • Necdin, a p53 target gene, regulates the quiescence and response to genotoxic stress of hematopoietic stem/progenitor cells.

    abstract::We recently defined a critical role for p53 in regulating the quiescence of adult hematopoietic stem cells (HSCs) and identified necdin as a candidate p53 target gene. Necdin is a growth-suppressing protein and the gene encoding it is one of several that are deleted in patients with Prader-Willi syndrome. To define th...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2011-11-393983

    authors: Asai T,Liu Y,Di Giandomenico S,Bae N,Ndiaye-Lobry D,Deblasio A,Menendez S,Antipin Y,Reva B,Wevrick R,Nimer SD

    更新日期:2012-08-23 00:00:00

  • Third-party CD4+ invariant natural killer T cells protect from murine GVHD lethality.

    abstract::Graft-versus-host disease (GVHD) is driven by extensive activation and proliferation of alloreactive donor T cells causing significant morbidity and mortality following allogeneic hematopoietic cell transplantation (HCT). Invariant natural killer T (iNKT) cells are a potent immunoregulatory T-cell subset in both human...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2014-11-612762

    authors: Schneidawind D,Baker J,Pierini A,Buechele C,Luong RH,Meyer EH,Negrin RS

    更新日期:2015-05-28 00:00:00

  • Applications of high-throughput DNA sequencing to benign hematology.

    abstract::The development of novel technologies for high-throughput DNA sequencing is having a major impact on our ability to measure and define normal and pathologic variation in humans. This review discusses advances in DNA sequencing that have been applied to benign hematologic disorders, including those affecting the red bl...

    journal_title:Blood

    pub_type: 杂志文章,评审

    doi:10.1182/blood-2013-07-460337

    authors: Sankaran VG,Gallagher PG

    更新日期:2013-11-21 00:00:00

  • Identification and characterization of a differentiation antigen in human neutrophils and monocytes.

    abstract::Human promyelocytic leukemia (HL-60) cells were induced to differentiate into macrophage-like cells by treatment with 10(-7) mol/L 1,25-dihydroxyvitamin D3 (VD3). A monoclonal antibody (MoAb, 60B8), reactive with the particulate of the differentiated cells but not of the untreated cells, was isolated. The antigen reco...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Nozawa R,Kato H,Ito T,Yokota T

    更新日期:1988-05-01 00:00:00

  • Update on clinical gene therapy for hemophilia.

    abstract::In contrast to other diverse therapies for the X-linked bleeding disorder hemophilia that are currently in clinical development, gene therapy holds the promise of a lasting cure with a single drug administration. Near-to-complete correction of hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficienc...

    journal_title:Blood

    pub_type: 杂志文章,评审

    doi:10.1182/blood-2018-07-820720

    authors: Perrin GQ,Herzog RW,Markusic DM

    更新日期:2019-01-31 00:00:00

  • CD34+ marrow cells, devoid of T and B lymphocytes, reconstitute stable lymphopoiesis and myelopoiesis in lethally irradiated allogeneic baboons.

    abstract::CD34+ cells devoid of detectable mature and immature T and B lymphocytes, expressing the CD2, CD10, and CD20 antigens, were isolated from marrows of three pairs of sex-mismatched, mixed lymphocyte culture (MLC) nonreactive, sibling baboons. Reciprocal transplants were performed between members of each pair, using the ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Andrews RG,Bryant EM,Bartelmez SH,Muirhead DY,Knitter GH,Bensinger W,Strong DM,Bernstein ID

    更新日期:1992-10-01 00:00:00

  • Interferon-beta interrupts interleukin-6-dependent signaling events in myeloma cells.

    abstract::Type I interferons (IFNs-alpha and IFN-beta) bind to a common receptor to exert strong antiproliferative activity on a broad range of cell types, including interleukin-6 (IL-6)-dependent myeloma cells. In this study, we investigated the effect of IFN-beta pretreatment on IL-6-stimulated mitogenic signaling in the huma...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Berger LC,Hawley RG

    更新日期:1997-01-01 00:00:00

  • Rh blood group-specific antibodies in immune hemolytic anemia induced by nomifensine.

    abstract::Nomifensine (Merital, Alival; Hoechst, Frankfurt, FRG), an antidepressant drug, may cause immune hemolytic anemia (IHA) of the so-called immune complex type that is believed to occur by means of an innocent-bystander mechanism. In this report we describe findings that are not consistent with this mechanism in a patien...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Salama A,Mueller-Eckhardt C

    更新日期:1986-12-01 00:00:00

  • Compartmentalization of cyclic GMP-dependent protein kinase in formyl-peptide stimulated neutrophils.

    abstract::The presence and physiologic role of cyclic GMP-dependent protein kinase (G-kinase) in human neutrophils was investigated by Western blot analysis and immunocytochemistry. Small quantities of G-kinase were found in the cytoskeletal-enriched fraction of neutrophil lysates as detected by Western blots using a polyclonal...

    journal_title:Blood

    pub_type: 杂志文章

    doi:

    authors: Pryzwansky KB,Wyatt TA,Nichols H,Lincoln TM

    更新日期:1990-08-01 00:00:00

  • Genomic characterization implicates iAMP21 as a likely primary genetic event in childhood B-cell precursor acute lymphoblastic leukemia.

    abstract::Intrachromosomal amplification of chromosome 21 (iAMP21) defines a distinct subgroup of childhood B-cell precursor acute lymphoblastic leukemia (BCP-ALL) that has a dismal outcome when treated with standard therapy. For improved diagnosis and risk stratification, the initiating genetic events need to be elucidated. To...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2011-01-329961

    authors: Rand V,Parker H,Russell LJ,Schwab C,Ensor H,Irving J,Jones L,Masic D,Minto L,Morrison H,Ryan S,Robinson H,Sinclair P,Moorman AV,Strefford JC,Harrison CJ

    更新日期:2011-06-23 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

  • Transduction of CLL cells by CD40 ligand enhances an antigen-specific immune recognition by autologous T cells.

    abstract::Several features of chronic lymphocytic leukemia (CLL) suggest that immune-based strategies may have therapeutic potential. A promising approach is provided by the transduction of CLL cells with CD40 ligand (CD40L) by viral vectors to enhance their immunogenicity. We compared the antigen-presenting capacity of CD40L-t...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2005-04-1742

    authors: Mayr C,Kofler DM,Büning H,Bund D,Hallek M,Wendtner CM

    更新日期:2005-11-01 00:00:00

  • K-Ras is essential for normal fetal liver erythropoiesis.

    abstract::In vitro studies suggest that Ras activation is necessary for erythroid cell development. However, genetic inactivation of the Ras isoforms H-Ras, N-Ras, and K-Ras in mice reportedly did not affect adult or fetal erythropoiesis, though K-Ras(-/-) embryos were anemic. Given these discrepancies, we performed a more deta...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2004-05-2021

    authors: Khalaf WF,White H,Wenning MJ,Orazi A,Kapur R,Ingram DA

    更新日期:2005-05-01 00:00:00

  • MRP8 and MRP14 control microtubule reorganization during transendothelial migration of phagocytes.

    abstract::MRP14 (S100A9) is the major calcium-binding protein of neutrophils and monocytes. Targeted gene disruption reveals an essential role of this S100 protein for transendothelial migration of phagocytes. The underlying molecular mechanism comprises major alterations of cytoskeletal metabolism. MRP14, in complex with its b...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood-2004-02-0446

    authors: Vogl T,Ludwig S,Goebeler M,Strey A,Thorey IS,Reichelt R,Foell D,Gerke V,Manitz MP,Nacken W,Werner S,Sorg C,Roth J

    更新日期:2004-12-15 00:00:00

  • An evolutionarily ancient mechanism for regulation of hemoglobin expression in vertebrate red cells.

    abstract::The oxygen transport function of hemoglobin (HB) is thought to have arisen ∼500 million years ago, roughly coinciding with the divergence between jawless (Agnatha) and jawed (Gnathostomata) vertebrates. Intriguingly, extant HBs of jawless and jawed vertebrates were shown to have evolved twice, and independently, from ...

    journal_title:Blood

    pub_type: 杂志文章

    doi:10.1182/blood.2020004826

    authors: Miyata M,Gillemans N,Hockman D,Demmers JAA,Cheng JF,Hou J,Salminen M,Fisher CA,Taylor S,Gibbons RJ,Ganis JJ,Zon LI,Grosveld F,Mulugeta E,Sauka-Spengler T,Higgs DR,Philipsen S

    更新日期:2020-07-16 00:00:00