Is sickle cell crisis a thrombotic event?

Abstract:

:Although large vessel thrombi are occasionally reported in patients with homozygous sickle cell disease, the role of intravascular coagulation in typical pain crises is controversial. Therefore, we studied 24 sickle cell patients during and between episodes of pain crisis, using several sensitive tests of hemostasis. Fibrinogen was measured by a clotting assay, beta-thromboglobulin (beta-TG) and fibrinopeptide A (FPA) were quantitated by radioimmunoassay, and protein C was determined by absorbing the zymogen from test plasma, activating it with thrombin-thrombomodulin complex, and measuring activity with a selective synthetic substrate. Fibrinogen was elevated in asymptomatic patients (355 +/- 145 mg/dl) but was no different from the value in these same patients during crisis (333 +/- 180 mg/dl, p greater than 0.1). Similarly, beta-TG 136 +/- 52 ng/ml vs 118 +/- 56; FPA 3.7 +/- 4.8 ng/ml vs 5.2 +/- 4.5, and protein C 71 +/- 20% vs 66 +/- 19 showed no important changes during crisis. However, all these values were significantly different from those in age- and sex-matched healthy controls. beta-TG, fibrinogen, and FPA were elevated (p less than 0.001, 0.005, and 0.05, respectively), and protein C was decreased (p less than 0.003). We conclude that while chronic intravascular coagulation is common in patients with sickle cell disease, there is no evidence that the pain crisis per se is a thrombotic event.

journal_name

Am J Hematol

authors

Green D,Scott JP

doi

10.1002/ajh.2830230403

subject

Has Abstract

pub_date

1986-12-01 00:00:00

pages

317-21

issue

4

eissn

0361-8609

issn

1096-8652

journal_volume

23

pub_type

杂志文章
  • Chimeric antigen receptor modified T cells that target chemokine receptor CCR4 as a therapeutic modality for T-cell malignancies.

    abstract::With the emerging success of treating CD19 expressing B cell malignancies with ex vivo modified, autologous T cells that express CD19-directed chimeric antigen receptors (CAR), there is intense interest in expanding this evolving technology to develop effective modalities to treat other malignancies including solid tu...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.24794

    authors: Perera LP,Zhang M,Nakagawa M,Petrus MN,Maeda M,Kadin ME,Waldmann TA,Perera PY

    更新日期:2017-09-01 00:00:00

  • Haploidentical transplantation outcomes for secondary acute myeloid leukemia: Acute Leukemia Working Party (ALWP) of the European Society for Blood and Marrow Transplantation (EBMT) study.

    abstract::Secondary acute myeloid leukemia (sAML) traditionally has inferior outcomes compared to de novo AML. Allogeneic hematopoietic cell transplantation (HCT) is the sole potentially curative therapy. This study analyzes the outcomes for unmanipulated haploidentical HCT (haploHCT) for sAML using the Acute Leukemia Working P...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.25087

    authors: Li Z,Labopin M,Ciceri F,Blaise D,Tischer J,Ehninger G,Van Lint MT,Koc Y,Santarone S,Forcade E,Castagna L,Polge E,Mailhol A,Ruggeri A,Mohty M,Savani BN,Nagler A

    更新日期:2018-06-01 00:00:00

  • Reversible posterior leukoencephalopathy syndrome complicating cytotoxic chemotherapy for hematologic malignancies.

    abstract::Reversible posterior leukoencephalopathy syndrome (RPLS) is an uncommon but distinctive clinicoradiological entity comprising of headache, seizures, visual disturbance, and altered mental function, in association with posterior cerebral white matter edema. With appropriate management, RPLS is reversible in the majorit...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20147

    authors: Tam CS,Galanos J,Seymour JF,Pitman AG,Stark RJ,Prince HM

    更新日期:2004-09-01 00:00:00

  • Feasibility, toxicity, and activity of LNH84-derived chemotherapy in the management of aggressive lymphomas.

    abstract::The results of chemotherapy remain unsatisfactory for many patients with advanced lymphomas. Both standard and more aggressive chemotherapy regimens might have their respective role in the management of theses diseases. We have tested the feasibility and assessed the toxicity and activity of a LNH84-derived chemothera...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1002/(sici)1096-8652(199707)55:4<199::aid-ajh6>

    authors: Laurencet FM,Zulian GB,Bauer J,Mermillod B,Iten PA,Alberto P

    更新日期:1997-08-01 00:00:00

  • Variation in the level of fetal hemoglobin in (delta beta) (0)-thalassemia heterozygotes with different numbers of alpha-globin genes.

    abstract::The Sicilian type of (delta beta) (0)-thalassemia characterized by a approximately 13 kb deletion, was present in a Turkish boy who is a homozygote and in his heterozygous parents who are first cousins. The father with approximately 21% Hb F had five alpha-globin genes (alpha alpha/alpha alpha alpha) and the mother wi...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830340316

    authors: Oner C,Gurgey A,Altay C,Kutlar F,Huisman TH

    更新日期:1990-07-01 00:00:00

  • The effect of budesonide mouthwash on oral chronic graft versus host disease.

    abstract::Oral chronic graft versus host disease (cGVHD) is common and a major cause of morbidity and loss of quality of life in long term survivors. Cyclosporine with prednisone remains the first line therapy for oral manifestations of cGVHD. However, even with routine administration of systemic agents, many patients with oral...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20814

    authors: Sari I,Altuntas F,Kocyigit I,Sisman Y,Eser B,Unal A,Fen T,Ferahbas A,Ozturk A,Unal A,Cetin M

    更新日期:2007-05-01 00:00:00

  • Durable molecular remissions with a single cycle of timed sequential consolidation chemotherapy in acute promyelocytic leukemia.

    abstract::In a pilot study to reduce the duration of treatment and potential long-term toxicities, 39 patients with acute promyelocytic leukemia in remission received a single cycle of intensive consolidation therapy, followed by intermittent ATRA maintenance. Consolidation therapy required prolonged hospitalization and was ass...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20354

    authors: Gore SD,Smith BD,Gojo I,Grever M,Kaufmann SH,Letendre L,Leonard DG,Marcucci G,Miller CB,Morris L,Piantadosi S,Prior T,Stock W,Karp JE

    更新日期:2005-06-01 00:00:00

  • Monocytic leukemia cutis diagnosed simultaneously with refractory anemia with monocytosis: a case report.

    abstract::A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. Skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing mon...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/1096-8652(200102)66:2<120::AID-AJH1027>3.0

    authors: Yavorkovsky LL,Zain J,Wu CD,Trivelli L,Cook P

    更新日期:2001-02-01 00:00:00

  • Sustained remissions and low rate of BCR-ABL resistance mutations with imatinib treatment chronic myelogenous leukemia in patients treated in late chronic phase: a 5-year follow up.

    abstract::The introduction of Imatinib (IM) has significantly altered the treatment for CML, although only limited follow-up results are available. As failure of Interferon-alpha had been associated with poor prognosis and results of IM-treatment in this patient group may allow earlier estimation of long-term benefits for early...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21055

    authors: Hess G,Meyer RG,Schuch B,Bechthold K,El-Kholy I,Huber C

    更新日期:2008-03-01 00:00:00

  • Demonstration of additional benefit in adding lenalidomide to azacitidine in patients with higher-risk myelodysplastic syndromes.

    abstract::Lenalidomide and azacitidine are active in MDS patients, and may complement each other by targeting the bone marrow microenvironment and the malignant clone. A recent Phase I trial testing the lenalidomide and azacitidine combination yielded encouraging results; however, lenalidomide’s contribution was unclear. In thi...

    journal_title:American journal of hematology

    pub_type: 杂志文章,多中心研究

    doi:10.1002/ajh.21891

    authors: Sekeres MA,O'Keefe C,List AF,Paulic K,Afable M 2nd,Englehaupt R,Maciejewski JP

    更新日期:2011-01-01 00:00:00

  • Hodgkin lymphoma: 2018 update on diagnosis, risk-stratification, and management.

    abstract:DISEASE OVERVIEW:Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8500 new patients annually and representing approximately 10.2% of all lymphomas in the United States. DIAGNOSIS:HL is composed of two distinct disease entities: classical HL and nodular lymphocyte predominant HL. Nodular sclero...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.25071

    authors: Ansell SM

    更新日期:2018-05-01 00:00:00

  • Human T-cell leukemia virus (HTLV-I) p24 antibody in New York City blood product recipients.

    abstract::Human T-cell leukemia virus (HTLV-I) is known to be associated with certain hematologic malignancies, and a related virus, HTLV-III/LAV, might be the cause of AIDS. Some persons with AIDS have had evidence of HTLV-I infection. Unrelated to these findings, it has been suggested that HTLV-I is transmitted via blood prod...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830200205

    authors: Jason JM,McDougal JS,Cabradilla C,Kalyanaraman VS,Evatt BL

    更新日期:1985-10-01 00:00:00

  • Refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS-T): 2017 update on diagnosis, risk-stratification, and management.

    abstract:DISEASE OVERVIEW:Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS-RS) and RARS with thromboc...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.24637

    authors: Patnaik MM,Tefferi A

    更新日期:2017-03-01 00:00:00

  • Mechanism for fetal hemoglobin induction by hydroxyurea in sickle cell erythroid progenitors.

    abstract::Hydroxyurea (HU) is a widely used cytotoxic agent that is known to induce fetal hemoglobin (HbF) production and is presently used to ameliorate the severity of pain episodes in patients with sickle cell anemia (HbSS). Previously we have shown that HU inhibits growth of burst forming unit-erythroid (BFU-E) colonies in ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/1096-8652(200011)65:3<227::aid-ajh9>3.0.co

    authors: Baliga BS,Pace BS,Chen HH,Shah AK,Yang YM

    更新日期:2000-11-01 00:00:00

  • The hope and reality of long-acting hemophilia products.

    abstract::Recombinant DNA technology and protein engineering are creating hope that we can address ongoing challenges in hemophilia care such as reducing the costs of therapy, increasing the availability to the developing world, and improving the functional properties of these proteins. Technological advances to improve the hal...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.23146

    authors: Pipe SW

    更新日期:2012-05-01 00:00:00

  • Passive donor-to-recipient transfer of antiphospholipid syndrome following allogeneic stem-cell transplantation.

    abstract::Autoantibody production following allogeneic stem-cell transplantation is common and is often ascribed to the immune dysregulation associated with graft-versus-host disease. Recent data suggests that donor-memory B cells can be reactivated on exposure to antigen and result in antibody production in the recipient ident...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20350

    authors: Ritchie DS,Sainani A,D'Souza A,Grigg AP

    更新日期:2005-08-01 00:00:00

  • Arsenic trioxide in the treatment of newly diagnosed acute promyelocytic leukemia: a single center experience.

    abstract::Arsenic trioxide (As(2)O(3)) has been found effective in the treatment in the treatment of acute promyelocytic leukemia (APML). Most studies with As(2)O(3) involve patients with APML who have relapsed following standard therapy. Between January 1998 and July 2000, 14 patients were recruited for an ongoing trial of As(...

    journal_title:American journal of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1002/ajh.10138

    authors: Mathews V,Balasubramanian P,Shaji RV,George B,Chandy M,Srivastava A

    更新日期:2002-08-01 00:00:00

  • Corticosteroids and rituximab as adjunctive treatments for thrombotic thrombocytopenic purpura.

    abstract::Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to fu...

    journal_title:American journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1002/ajh.23126

    authors: George JN

    更新日期:2012-05-01 00:00:00

  • Utility and prognostic value of 18 F-FDG positron emission tomography-computed tomography scans in patients with newly diagnosed multiple myeloma.

    abstract::Positron emission tomography-computed tomography (PET-CT) can identify bony lesions, assess disease burden, and detect extramedullary disease (EMD) in patients with multiple myeloma. We retrospectively reviewed records of patients who underwent PET-CT within 60 days of a new diagnosis (before therapy commenced) to ide...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.25279

    authors: Aljama MA,Sidiqi MH,Buadi FK,Lacy MQ,Gertz MA,Dispenzieri A,Dingli D,Muchtar E,Fonder AL,Hayman SR,Hobbs MA,Gonsalves WI,Warsame RM,Kourelis T,Hwa YL,Kapoor P,Kyle RA,Leung N,Go RS,Rajkumar SV,Kumar SK

    更新日期:2018-12-01 00:00:00

  • Venous thromboembolism prophylaxis in medically ill patients and the development of strategies to improve prophylaxis rates.

    abstract::Venous thromboembolism (VTE) is common but often unrecognized in medically ill patients. Over the past 5 years, three large-scale placebo-controlled trials enrolling a total of 5500 medically ill patients have highlighted the risk of VTE in this group. These trials have helped to define a specific at-risk patient prof...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20281

    authors: Stinnett JM,Pendleton R,Skordos L,Wheeler M,Rodgers GM

    更新日期:2005-03-01 00:00:00

  • Cell fusion is not involved in the generation of giant cells in the Hodgkin-Reed Sternberg cell line L1236.

    abstract::The mechanism of multinucleated cell formation in Hodgkin's disease has not yet been elucidated. We asked whether the giant multinucleated cells of the H-RS cell line L1236 develop via fusion of the predominant smaller cells. As a positive control for the fusion assay, human B cells from the B-cell lymphoma cell line ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.1068

    authors: Re D,Benenson E,Beyer M,Gresch O,Draube A,Diehl V,Wolf J

    更新日期:2001-05-01 00:00:00

  • Hemostatic molecular markers before the onset of disseminated intravascular coagulation.

    abstract::We retrospectively measured various hemostatic markers in 240 patients with disseminated intravascular coagulation (DIC) before the onset of DIC and in 110 non-DIC patients, and examined their usefulness for the diagnosis of pre-DIC. Changes in prothrombin time ratio and fibrinogen levels were not significant before t...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/(sici)1096-8652(199904)60:4<273::aid-ajh4>

    authors: Wada H,Sakuragawa N,Mori Y,Takagi M,Nakasaki T,Shimura M,Hiyoyama K,Nisikawa M,Gabazza EC,Deguchi K,Kazama M,Shiku H

    更新日期:1999-04-01 00:00:00

  • UGT2B7 promoter variant -840G>A contributes to the variability in hepatic clearance of morphine in patients with sickle cell disease.

    abstract::The purpose of the study was to determine if UDP-glucuronosyltransferase (UGT) 2B7 allelic variants encoding for UGT2B7, primary enzyme responsible for morphine glucuronidation contribute to the variability in the hepatic clearance of morphine in sickle cell disease (SCD). Twenty-four hour PK study of morphine and UGT...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.21051

    authors: Darbari DS,van Schaik RH,Capparelli EV,Rana S,McCarter R,van den Anker J

    更新日期:2008-03-01 00:00:00

  • Rearrangement and expression of MDM2 oncogene in chronic lymphocytic leukemia.

    abstract::Since trisomy 12 is the most common chromosome abnormality found in CLL and MDM2 has been mapped to this chromosome, we examined the possible association of MDM2 in the pathogenesis of CLL. A rearrangement of the MDM2 gene was observed in 4 of 11 peripheral blood mononuclear cells (PBMC) from patients with CLL by Sout...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830470215

    authors: Huang YQ,Raphael B,Buchbinder A,Li JJ,Zhang WG,Friedman-Kien AE

    更新日期:1994-10-01 00:00:00

  • Humoral immune responses to gag and env proteins from human immunodeficiency virus type 1 in hemophiliac patients.

    abstract::Solid-phase enzyme immunoassays using recombinant gag and env proteins were developed to study humoral immune responses to HIV infection in a cohort of 105 hemophiliac patients. Thirteen patients with ARC or AIDS and 92 asymptomatic patients were studied. A cross-sectional study showed a wide range of antibody respons...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830360108

    authors: Kinney JS,Conway JH,Hilgartner MW,Clayman B,Mayur K,Yolken RH,Viscidi RP

    更新日期:1991-01-01 00:00:00

  • Erythrocytes as carriers of chemotherapeutic agents for targeting the reticuloendothelial system.

    abstract::The object of this work was to define a model using hypotonically loaded erythrocytes as a vehicle to target drugs to the reticuloendothelial system (RES). The optimum hemolytic event was found to occur at 100 mOsm/kg using a 0.5-min exposure at 0 degrees C. Approximately one third of the total volume of the cells cou...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830090303

    authors: Lynch WE,Sartiano GP,Ghaffar A

    更新日期:1980-01-01 00:00:00

  • Prevalence of pulmonary hypertension in hereditary spherocytosis.

    abstract::Vascular complications, including pulmonary hypertension (PH), have been reported to occur following splenectomy for various disorders,including hereditary spherocytosis (HS). We performed a prospective cross-sectional study of 36 adults with HS (78% with prior splenectomy)utilizing echocardiography to estimate tricus...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.22182

    authors: Crary SE,Ramaciotti C,Buchanan GR

    更新日期:2011-12-01 00:00:00

  • Prognostic factors for therapeutic outcome of diffuse small non-cleaved cell lymphoma in adults.

    abstract::Most reports of prognosis and therapy in diffuse small non-cleaved cell lymphoma (DSNCL), an aggressive high-grade non-Hodgkin's lymphoma (NHL) which appears to be of two histopathologic subtypes, have included predominantly a pediatric population and very few adults. We studied 20 newly diagnosed, previously untreate...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830460408

    authors: Morrison VA,Frizzera G,Arthur DC,Ogle KM,Hurd DD,Bloomfield CD,Peterson BA

    更新日期:1994-08-01 00:00:00

  • Sickle cell anemia and beta-gene cluster haplotypes in Cuba.

    abstract::We have studied 91 patients with SS genotype, 44 children and 47 adults. Excluding the Cameroon and atypical haplotypes, the distribution in the children's sample exhibited 43% Benin, 38% Bantu, and 3% Senegal. In adults, the sample exhibited 46% Benin, 30% Bantu, and 9% Senegal (chi 2: 13.511, 2 df, P = 0.001). When ...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.2830490212

    authors: Muniz A,Corral L,Alaez C,Svarch E,Espinosa E,Carbonell N,di Leo R,Felicetti L,Nagel RL,Martinez G

    更新日期:1995-06-01 00:00:00

  • Warfarin reversal emerging as the major indication for fresh frozen plasma use at a tertiary care hospital.

    abstract::Because of the increase in the use of warfarin in the population in recent years, reversal of warfarin-related coagulopathy has become common in daily hospital practice. Transfusion of fresh frozen plasma (FFP) is the preferred treatment method for urgent warfarin reversal in the USA. We have undertaken a 1-month audi...

    journal_title:American journal of hematology

    pub_type: 杂志文章

    doi:10.1002/ajh.20902

    authors: Ozgonenel B,O'Malley B,Krishen P,Eisenbrey AB

    更新日期:2007-12-01 00:00:00