Disturbance of plasma and platelet thrombospondin levels in sickle cell disease.


:Thrombospondin (TSP), a large protein found in platelet alpha-granules (as TSP-1), mediates adhesion of sickle reticulocytes to cultured vascular endothelium. To further explore the physiologic relevance of this observation, we have measured plasma TSP levels and platelet TSP-1 content in subjects with sickle cell disease. Plasma TSP levels were similar for normal controls (mean 491 ng/ml, range 331-723) and steady-state HbSS patients (mean 536, range 333-1107) and were significantly (P = 0.012) but variably elevated for HbSS patients presenting with acute painful crisis (mean 868, range 442-2780). Some of these elevated plasma TSP levels reached those previously observed to support maximal red cell adhesion to endothelium in vitro. Compared to normals, both steady-state and in-crisis HbSS patients had significantly (P < 0.001) depressed platelet TSP-1 content (82.6 +/- 11.9, 47.1 +/- 16.0 and 45.9 +/- 20.7 ng/l0(6) platelets, respectively, mean +/- SD). HbSC disease patients, all examined during steady state, had low-normal plasma levels of TSP and either normal or depressed platelet TSP-1 content. Serial observations on three sickle cell anemia subjects indicated a probable relationship between platelet TSP-1 release, elevated plasma TSP levels, and acute vaso-occlusive episodes. These results suggest a state of ongoing release and depletion of TSP-1 from activated platelets in patients with sickle cell disease.


Am J Hematol


Browne PV,Mosher DF,Steinberg MH,Hebbel RP




Has Abstract


1996-04-01 00:00:00














  • Peripheral blood remission of hairy cell leukemia after transfusion hepatitis.

    abstract::Hairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and ...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Keefer MJ,Weber MJ,Bottomley SS,Solanki DL,Hosty TA

    更新日期:1987-07-01 00:00:00

  • CD7 expression on CD34+ cells from chronic myeloid leukaemia in chronic phase.

    abstract::Thirty-seven patients with chronic phase chronic myeloid leukaemia and fourteen healthy controls have been evaluated for lineage differentiation with immunological markers on purified bone marrow CD34 positive cells by multiparameter flow cytometry. The myeloid-associated antigen CD33 and the stem cell factor receptor...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Martín-Henao GA,Quiroga R,Sureda A,García J

    更新日期:1999-07-01 00:00:00

  • Gene therapies for transfusion dependent β-thalassemia: Current status and critical criteria for success.

    abstract::Thalassemia is one of the most prevalent monogenic diseases usually caused by quantitative defects in the production of β-globin leading to severe anemia. Technological advances in genome sequencing, stem cell selection, viral vector development, transduction and gene editing strategies now allow for efficient exvivo ...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审


    authors: Soni S

    更新日期:2020-09-01 00:00:00

  • Retraction: 'Number needed to treat with 4-factor prothrombin complex concentrate for urgent warfarin reversal' by Andrew Chua, Vishal Patel, Allison Perrin, Lee Stern, Jenifer Ehreth, Laurel Omert, Christopher Hood, Julie Farley, Michael McGlynn and Lipi

    abstract::The above abstract from the THSNA 2016 Summit Abstract Proceedings, first published online in the American Journal of Hematology on 20 July 2016 in Wiley OnlineLibrary (www.onlinelibrary.wiley.com), and in Volume 91, Issue 9, p. E427, has been retracted by agreement between the authors, the journal Editor-in-Chief, Ca...

    journal_title:American journal of hematology

    pub_type: 杂志文章,撤回出版物



    更新日期:2017-04-01 00:00:00

  • Autoimmune hemolytic anemia in an infant with giant cell hepatitis.

    abstract::Autoimmune hemolytic anemia (AIHA) associated with giant cell hepatitis (GCH) is a rare disorder in infants. AIHA usually precedes the development of liver disease by months to years. Early recognition of the disease and prompt institution of immunosuppressive therapy results in clinical remission and prevents liver d...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Kashyap R,Sarangi JN,Choudhry VP

    更新日期:2006-03-01 00:00:00

  • Langerhans cell histiocytosis: lack of a viral etiology.

    abstract::Langerhans cell histiocytosis (LCH) is an enigmatic histiocytic proliferative disorder of unknown etiology that affects children primarily. We have investigated the possibility that viruses are etiological or that they have a "triggering effect" in LCH. Sensitive in situ hybridization and polymerase chain reaction (PC...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: McClain K,Jin H,Gresik V,Favara B

    更新日期:1994-09-01 00:00:00

  • Granulocytes from chronic myeloid leukemia (CML) patients show differential response to different chemoattractants.

    abstract::Binding of chemoattractant to polymorphonuclear leukocytes (PMNL) triggers a series of events like polymerization of actin and tubulin, orientation of cells, chemotaxis, increase in fluid pinocytosis and phagocytosis, and stimulation of microbicidal pathways which includes lysosomal degranulation and generation of rea...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Radhika V,Thennarasu S,Naik NR,Kumar A,Advani SH,Bhisey AN

    更新日期:1996-07-01 00:00:00

  • Single agents vs combination chemotherapy in relapsed and refractory peripheral T-cell lymphoma: Results from the comprehensive oncology measures for peripheral T-cell lymphoma treatment (COMPLETE) registry.

    abstract::Single agents have demonstrated activity in relapsed and refractory (R/R) peripheral T-cell lymphoma (PTCL). Their benefit relative to combination chemotherapy remains undefined. Patients with histologically confirmed PTCL were enrolled in the Comprehensive Oncology Measures for Peripheral T-cell Lymphoma Treatment (C...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Stuver RN,Khan N,Schwartz M,Acosta M,Federico M,Gisselbrecht C,Horwitz SM,Lansigan F,Pinter-Brown LC,Pro B,Shustov AR,Foss FM,Jain S

    更新日期:2019-06-01 00:00:00

  • Pure red cell aplasia with thymona: evidence of T-cell clonal disorder.

    abstract::Pure red cell aplasia (PRCA) sometimes accompanies thymoma. Herein, we report a PRCA patient with thymoma with a clonal disorder of T cells. A 55-year-old man presented with anemia and anterior mediastinum tumor. The laboratory study revealed hemoglobin 8.2 g/dl; leukocytes 15.8 x 10(9)/L with 76.5% neutrophils, 20.0%...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Masuda M,Arai Y,Okamura T,Mizoguchi H

    更新日期:1997-04-01 00:00:00

  • Genotypic analysis using a Y-chromosome-specific probe following bone marrow transplantation.

    abstract::To monitor successful engraftment after bone marrow transplantation, we performed Southern hybridization analysis or dot blot analysis of DNA in a set of sex-mismatched cases using a Y-chromosome-specific DNA probe (pHY10). This method was extremely sensitive and rapid for checking which cells contain the Y-chromosome...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Morisaki H,Morisaki T,Nakahori Y,Ogura H,Kanno H,Tani K,Kodo H,Fujii H,Asano S,Miwa S

    更新日期:1988-01-01 00:00:00

  • Exposure to phorbol diester (TPA) in vitro as an aid in the classification of blasts in human myelogenous and lymphoid leukemias: in vitro differentiation, growth patterns, and ultrastructural observations.

    abstract::Leukemic cells from the peripheral blood of 52 patients with acute and chronic leukemias were incubated with 12-0-tetradecanoyl phorbol ester (TPA). Thirty-one cases of lymphocytic leukemia (18 cases of acute lymphoblastic and 13 cases of chronic lymphocytic leukemia), 13 cases of acute nonlymphoblastic (myelo or myel...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Polliack A,Leizerowitz R,Korkesh A,Gurfel D,Gamliel H,Galili U

    更新日期:1982-11-01 00:00:00

  • Splenic lymphoma with circulating villous lymphocytes: report of seven cases and review of the literature.

    abstract::Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new entity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic studies to further characterize this lymphoma, which is frequently confused with hairy cell leukemia and other ...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审


    authors: Sun T,Susin M,Brody J,Dittmar K,Teichberg S,Weiner R,Lin JH,Felber N

    更新日期:1994-01-01 00:00:00

  • Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia.

    abstract::Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years be...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Vicari P,Barretto de Mello A,Figueiredo MS

    更新日期:2005-03-01 00:00:00

  • B-cell chronic lymphocytic leukaemia: prognostic value of the immunophenotype and the clinico-haematological features.

    abstract::Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Orfao A,Gonzalez M,San Miguel JF,Rios A,Canizo MC,Hernandez J,Maricato ML,Lopez Borrasca A

    更新日期:1989-05-01 00:00:00

  • Abnormal gamma IFN and alpha TNF secretion in purified CD2+ cells from autoimmune thrombocytopenic purpura (ATP) patients: their implication in the clinical course of the disease.

    abstract::Gamma inferferon (gamma IFN), alpha tumor necrosis factor (alpha TNF), and interleukin 6 (IL-60) are cytokines produced by a wide variety of cells, including T lymphocytes and NK cells. These cytokines affect B-cell proliferation and differentiation into immunoglobulin secreting cells. In addition, gamma IFN and alpha...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Garcia-Suarez J,Prieto A,Reyes E,Manzano L,Arribalzaga K,Alvarez-Mon M

    更新日期:1995-08-01 00:00:00

  • Myelodysplastic transformation of polycythemia vera: case report and review of the literature.

    abstract::We report a case of refractory anemia with excess blasts (RAEB) developing in a 67-year old man with a history of polycythemia vera; results of cytogenetic and immunophenotyping studies are described. In this report the clinical, cytogenetic and hematologic features of myelodysplasia complicating polycythemia vera are...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审


    authors: Shamdas GJ,Spier CM,List AF

    更新日期:1991-05-01 00:00:00

  • Familial type II protein C deficiency associated with warfarin-induced skin necrosis and bilateral adrenal hemorrhage.

    abstract::A family is described in which venous thromboembolic disease is associated with reduced plasma protein C activity and normal levels of protein C antigen. Immunoelectrophoretic analysis of protein C antigen gave an abnormal pattern in all affected members, suggesting that the disorder is related to the presence of a st...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Conlan MG,Bridges A,Williams E,Marlar R

    更新日期:1988-12-01 00:00:00

  • Mutant calreticulin-expressing cells induce monocyte hyperreactivity through a paracrine mechanism.

    abstract::Mutations in the calreticulin gene (CALR) were recently identified in approximately 70-80% of patients with JAK2-V617F-negative essential thrombocytosis and primary myelofibrosis. All frameshift mutations generate a recurring novel C-terminus. Here we provide evidence that mutant calreticulin does not accumulate effic...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Garbati MR,Welgan CA,Landefeld SH,Newell LF,Agarwal A,Dunlap JB,Chourasia TK,Lee H,Elferich J,Traer E,Rattray R,Cascio MJ,Press RD,Bagby GC,Tyner JW,Druker BJ,Dao KH

    更新日期:2016-02-01 00:00:00

  • Laboratory evaluation of clopidogrel responsiveness by platelet function and genetic methods.

    abstract::Clopidogrel is a widely used antiplatelet agent that irreversibly inhibits platelet P2Y12 ADP receptors after conversion to an active metabolite. There are a number of laboratory tests capable of detecting clopidogrel-induced platelet inhibition and published literature correlates suboptimal clopidogrel response to ad...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审


    authors: Smock KJ,Saunders PJ,Rodgers GM,Johari V

    更新日期:2011-12-01 00:00:00

  • New alpha 2 globin chain variant with low oxygen affinity affecting the N-terminal residue and leading to N-acetylation [Hb Lyon-Bron alpha 1(NA1)Val --> Ac-Ala].

    abstract::Hemoglobin Lyon-Bron was found in two members of a family of German ascent presenting with a moderate normocytic anemia. In this alpha 2 globin variant, the N-terminal valine of the chain was replaced by an alanine. Electrospray mass spectrometry of the alpha chain showed that, as normally, the initiator methionine wa...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Lacan P,Souillet G,Aubry M,Promé D,Richelme-David S,Kister J,Wajcman H,Francina A

    更新日期:2002-03-01 00:00:00

  • Phase I study of oral clofarabine consolidation in adults aged 60 and older with acute myeloid leukemia.

    abstract::Clofarabine has shown activity and tolerability in older patients with acute myeloid leukemia (AML). We investigated the safety and tolerability of an oral formulation of clofarabine for consolidation therapy of patients aged 60 and older with AML. In this phase I study, twenty-two patients older than 60 years with AM...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Jacoby MA,Martin MG,Uy GL,Westervelt P,Dipersio JF,Cashen A,Stockerl-Goldstein K,Vij R,Luo J,Reineck T,Bernabe N,Abboud CN

    更新日期:2014-05-01 00:00:00

  • T-gamma large granular lymphocyte leukemia associated with amegakaryocytic thrombocytopenic purpura, Sjögren's syndrome, and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia.

    abstract::We present a female patient with T-gamma LGL leukemia, who was followed for the last 20 years. Over these years she developed several autoimmune disorders, including Sjögren's syndrome, Hashimoto's thyroiditis, premature ovarian failure (compatible with type II autoimmune polyglandular syndrome), amegakaryocytic throm...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Ergas D,Tsimanis A,Shtalrid M,Duskin C,Berrebi A

    更新日期:2002-02-01 00:00:00

  • Detection of monosomy 7 in interphase cells of patients with myeloid disorders.

    abstract::Six patients, five with acute myeloid leukemia (AML) and one with a myelodysplastic syndrome (MDS), were found to have monosomy 7 by conventional cytogenetics at diagnosis. Repetitive DNA sequences from the heterochromatic region of human chromosomes 1 and 7 were used as probes for in situ hybridization experiments on...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Kolluri RV,Manuelidis L,Cremer T,Sait S,Gezer S,Raza A

    更新日期:1990-02-01 00:00:00

  • Late appearance of the Philadelphia chromosome with monosomy 7 in a patient with de novo AML with trilineage myelodysplasia.

    abstract::We report here a patient with de novo acute myelocytic leukemia with trilineage myelodysplasia (AML/TMDS) in whom cytogenetic analysis was normal at diagnosis and in whom Philadelphia chromosome (Ph1) with monosomy 7 emerged at the terminal stage of the disease. Reverse transcription polymerse chain reaction (RT-PCTR)...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Matsue K,Miyamoto T,Ito M,Tsukuda K

    更新日期:1995-08-01 00:00:00

  • Effect of heparin on platelet aggregation.

    abstract::The effect of heparin on platelet aggregation was systematically examined on platelets in plasma (PRP), as well as on gel-filtered, washed, and formaldehyde-fixed platelets. Results indicate that, although heparin causes a mild potentiation of platelet aggregation in the PRP systems, a significant inhibitory activity ...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Saba HI,Saba SR,Morelli GA

    更新日期:1984-10-01 00:00:00

  • Nongastric marginal zone B-cell lymphoma: analysis of 247 cases.

    abstract::Nongastric marginal zone B-cell lymphoma (NG-MZL) is a relatively uncommon indolent lymphoma. From 1990 to 2005, a total of 247 patients with histologically confirmed NG-MZL were analyzed. Ann Arbor stage I/II disease was present in 78% (167 out of 215). One hundred eighty-six patients out of two hundred eight were ca...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Oh SY,Ryoo BY,Kim WS,Park YH,Kim K,Kim HJ,Kwon JM,Lee J,Ko YH,Ahn YC,Oh SJ,Lee SI,Kim HJ,Kwon HC,Bang SM,Kim JH,Park J,Lee SS,Kim HY,Park K

    更新日期:2007-06-01 00:00:00

  • Free immunoglobulin light-chain serum levels in the follow-up of patients with monoclonal gammopathies: correlation with 24-hr urinary light-chain excretion.

    abstract::In patients with light-chain myeloma or primary AL-amyloidosis, 24-hr light-chain excretion in the urine is considered an essential marker of the tumor mass. However, 24-hr urine collection and analysis may be cumbersome and prone to inaccuracy. Recently, a sensitive immunonephelometric assay for immunoglobulin free l...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Alyanakian MA,Abbas A,Delarue R,Arnulf B,Aucouturier P

    更新日期:2004-04-01 00:00:00

  • On the temporal development of erythrocyte sedimentation rate using sealed vacuum tubes.

    abstract::The temporal development of the erythrocyte sedimentation rate (ESR) was studied in wide, short vacuum tubes. It was found that in about 3% of the specimens arriving in the laboratory the ESR developed in three different phases during 60 min, whereas the other showed only two. The specimens with three phases behaved s...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Kallner A

    更新日期:1991-07-01 00:00:00

  • Erythrocyte pyruvate kinase deficiency: a kinetic method for differentiation between heterozygosity and compound-heterozygosity.

    abstract::The goal of the present study was to search for criteria that allow one to distinguish between normal individuals and heterozygotes as well as compound heterozygotes for pyruvate kinase (PK) deficiency. As the residual activity of PK with heterozygotes was between 35% and 110% of the normal activity, it was necessary ...

    journal_title:American journal of hematology

    pub_type: 杂志文章


    authors: Lakomek M,Winkler H,Linne S,Schröter W

    更新日期:1989-08-01 00:00:00

  • The impact of cytogenetic risk on the outcomes of allogeneic hematopoietic cell transplantation in patients with relapsed/refractory acute myeloid leukemia: On behalf of the acute leukemia working party (ALWP) of the European group for blood and marrow tr

    abstract::Karyotypic analysis at time of diagnosis has an important value in determining initial response to treatment, remission duration and overall survival (OS) in acute myeloid leukemia (AML). Less is known about its value before allogeneic hematopoietic cell transplantation (allo-HCT) in patients transplanted with active ...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Poiani M,Labopin M,Battipaglia G,Beelen DW,Tischer J,Finke J,Brecht A,Forcade E,Ganser A,Passweg JR,Labussiere-Wallet H,Yakoub-Agha I,Schäfer-Eckart K,Kroeger N,Guffroy B,Ruggeri A,Esteve J,Nagler A,Mohty M

    更新日期:2021-01-01 00:00:00