Protein C system defects in Indian children with thrombosis.

Abstract:

:As ethnic variations are known to exist in inherited genetic defects, the clinico-haematological profile of Indian children with thrombophilia may be different from that of Caucasians. The aim of the study was to analyse the phenotypic and genotypic causes of thrombophilia in Indian children. Forty patients with arterial (21 patients) and venous (19 patients) thrombosis were the subjects of the study. Their age ranged from 6 days to 15 years. All of the patients were initially screened by Pro C Global assay. Activated protein C resistance (APCR) was measured. In cases with low Pro C Global values, protein C (PC), protein S (PS) and factor V G1691A, prothrombin G20210A and MTHFR C677T polymorphism were tested in all 40 cases. Of the 21 patients with arterial thrombosis, 4 (19%) had PC deficiency, 7 (33.3%) had PS deficiency and 1 (4.8%) had combined deficiency of PC and PS. Of the 19 patients with venous thrombosis, 5 (26.3%) each had PC and PS deficiency and 4 (21%) had combined PC and PS deficiency. Heterozygous factor V G1691A defect was seen in one (4.8%) patient with arterial thrombosis and three (15.8%) patients with venous thrombosis. Heterozygous MTHFR C677T polymorphism was seen in five (23.8%) patients with arterial thrombosis and in four (21%) patients with venous thrombosis. Prothrombin G20210A polymorphism was absent in all patients and controls. Protein C system defect is common in Indian children with thrombosis.

journal_name

Ann Hematol

journal_title

Annals of hematology

authors

Gupta PK,Ahmed RP,Bhattacharyya M,Kannan M,Biswas A,Kalra V,Saxena R

doi

10.1007/s00277-004-0952-x

subject

Has Abstract

pub_date

2005-02-01 00:00:00

pages

85-8

issue

2

eissn

0939-5555

issn

1432-0584

journal_volume

84

pub_type

杂志文章
  • Influence of conditioning regimens and stem cell sources on donor-type chimerism early after stem cell transplantation.

    abstract::We retrospectively analyzed very early chimerism before and ongoing neutrophil engraftment (days 7, 14, 21, 28) and investigated the influence of conditioning regimens and stem cell sources on donor-type chimerism in 59 Japanese patients who had received allogeneic hematopoietic stem cell transplantation. The percenta...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-008-0542-4

    authors: Sugita J,Tanaka J,Hashimoto A,Shiratori S,Yasumoto A,Wakasa K,Kikuchi M,Shigematsu A,Miura Y,Tsutsumi Y,Kondo T,Asaka M,Imamura M

    更新日期:2008-12-01 00:00:00

  • Impact of clinical features, cytogenetics, genetic mutations, and methylation dynamics of CDKN2B and DLC-1 promoters on treatment response to azacitidine.

    abstract::Azacitidine (AZA) is a DNA hypomethylation agent administered in myeloid neoplasms; however, there is still a lack of established predictors of response. We studied 113 patients with myelodysplastic syndromes (n = 85) or acute myeloid leukemia (n = 28) who received AZA to assess the predictive value on response of cli...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1007/s00277-020-03932-8

    authors: Martín I,Navarro B,Serrano A,Villamón E,Calabuig M,Solano C,Chaves FJ,Yagüe N,Orts M,Amat P,Fuentes A,Seda E,García F,Hernández-Boluda JC,Tormo M

    更新日期:2020-03-01 00:00:00

  • A phase 1 clinical trial of flavopiridol consolidation in chronic lymphocytic leukemia patients following chemoimmunotherapy.

    abstract::Patients with chronic lymphocytic leukemia (CLL) who receive chemoimmunotherapy and do not achieve complete remission experience significantly shortened progression-free interval (PFS). Additionally, the majority of patients treated for relapsed disease demonstrate evidence of measurable disease. Eradication of minima...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1007/s00277-016-2683-1

    authors: Awan FT,Jones JA,Maddocks K,Poi M,Grever MR,Johnson A,Byrd JC,Andritsos LA

    更新日期:2016-06-01 00:00:00

  • Low-density lipoprotein (LDL) levels and risk of arterial occlusive events in chronic myeloid leukemia patients treated with nilotinib.

    abstract::Recommendations for dyslipidemia management aimed at reducing arterial occlusive events (AOEs) have been recently published. So far, no data have been reported on the management of dyslipidemia in chronic myeloid leukemia (CML) patients treated with nilotinib. We investigated 369 CML adult patients, stratified accordi...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-020-04392-w

    authors: Caocci G,Mulas O,Capodanno I,Bonifacio M,Annunziata M,Galimberti S,Luciano L,Tiribelli M,Martino B,Castagnetti F,Binotto G,Pregno P,Stagno F,Abruzzese E,Bocchia M,Gozzini A,Albano F,Fozza C,Luzi D,Efficace F,Simul

    更新日期:2021-01-03 00:00:00

  • Allogeneic peripheral blood stem cell transplantation following fludarabine-based conditioning in six children with advanced Hodgkin's disease.

    abstract::Despite high-dose chemotherapy and autografting, the outcome for patients with primary refractory Hodgkin's disease (HD) or multiple relapses remains unsatisfactory. Six pediatric patients (median age: 16 years, range: 11-19) received reduced intensity conditioning and allogeneic peripheral blood stem cell transplanta...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-003-0814-y

    authors: Claviez A,Klingebiel T,Beyer J,Nürnberger W,Ehninger G,Suttorp M,Dreger P,Dörffel W,Schmitz N

    更新日期:2004-04-01 00:00:00

  • Anaphylactoid reaction with bronchospasm following intravenous cyclophosphamide administration.

    abstract::Anaphylactoid reaction to cyclophosphamide administration in a 85-year old woman is described. Symptomatology was restricted to bronchospasm, which regressed within one hour. This incomplete and rapidly regressive symptomatology might be explained by previous administration of methylprednisolone. No anaphylactoid symp...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/BF01714904

    authors: Salles G,Vial T,Archimbaud E

    更新日期:1991-02-01 00:00:00

  • In vitro response of blasts to IL-3, GM-CSF, and G-CSF is different for individual AML patients: factors that stimulate leukemic clonogenic cells also enhance Ara-C cytotoxicity.

    abstract::In vivo, growth factors are currently investigated for their capacity to trigger leukemic stem cells into cycle and thus overcome kinetic drug resistance. In this study, the susceptibility of leukemic clonogenic cells to individual growth factors was related to cytosine-arabinoside (Ara-C) sensitivity. The effects of ...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/BF01737421

    authors: Van der Lely N,De Witte T,Wessels J,Raymakers R,Muus P,Preijers F

    更新日期:1994-05-01 00:00:00

  • Severe bleeding in two patients due to increased sensitivity of factor IX activity to phenprocoumon therapy.

    abstract::Two male patients with severe and recurrent bleeding episodes under phenprocoumon therapy are reported. Both patients exhibited a strong decrease of their factor IX activities below 1% of normal, whereas the activities of the vitamin K-dependent factors prothrombin, VII, and X were found to be within or above the expe...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s002770050297

    authors: Quenzel EM,Hertfelder HJ,Oldenburg J

    更新日期:1997-06-01 00:00:00

  • PD-L1, LAG3, and HLA-DR are increasingly expressed during smoldering myeloma progression.

    abstract::Symptomatic multiple myeloma (MM) is a plasma cell neoplasm that represents the final stage of a continuum of clinical conditions that start from monoclonal gammopathy of unknown significance (MGUS), then transits in the more advance, but still asymptomatic, smoldering MM (SMM), with a final evolution in symptomatic M...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1007/s00277-019-03648-4

    authors: Mussetti A,Pellegrinelli A,Cieri N,Garzone G,Dominoni F,Cabras A,Montefusco V

    更新日期:2019-07-01 00:00:00

  • Haemoglobinopathies with high oxygen affinity. Experience of Erythropathology Cooperative Spanish Group.

    abstract::Haemoglobinopathies are the world's most frequently found monogenic disorders. In the cases with high oxygen affinity, the decrease in the liberation of the oxygen determines a secondary erythrocytosis. In this work, we present 17 unrelated families of Caucasian race and of Spanish origin, with ten variants of haemogl...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-008-0581-x

    authors: González Fernández FA,Villegas A,Ropero P,Carreño MD,Anguita E,Polo M,Pascual A,Henández A

    更新日期:2009-03-01 00:00:00

  • Intercellular adhesion molecular 1 on monocytes mediates adhesion as well as trans-endothelial migration and can be downregulated using antisense oligonucleotides.

    abstract::The intercellular adhesion molecule 1 (ICAM-1) on endothelial cells is involved in the recruitment of leukocytes to inflammatory sites. In contrast to ICAM-1 expression on endothelial cells, little is known about its function in leukocytes in inflammation. Using ICAM-1-directed anti-sense oligodeoxyribonucleotides (OD...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s002770000168

    authors: Steidl U,Haas R,Kronenwett R

    更新日期:2000-08-01 00:00:00

  • Erythropoiesis-stimulating agents in the treatment of anemia in myelodysplastic syndromes: a meta-analysis.

    abstract::The present meta-analysis was undertaken to (1) assess erythroid response rates in myelodysplastic syndromes (MDS) patients treated with epoetin alfa as a monotherapy, (2) gain further insights into predictors of response rates, and (3) compare the erythroid response rates observed with epoetin alfa and darbepoetin al...

    journal_title:Annals of hematology

    pub_type: 杂志文章,meta分析,评审

    doi:10.1007/s00277-008-0450-7

    authors: Moyo V,Lefebvre P,Duh MS,Yektashenas B,Mundle S

    更新日期:2008-07-01 00:00:00

  • Hematopoietic growth factors and the treatment of tumor-associated anemias.

    abstract::Erythropoietin, alone or in combination with colony-stimulating factors, is a promising agent in the treatment of patients with cancer-related 'anemia of chronic disorders', chemo/radiotherapy-induced anemia, or anemia due to myelodysplastic or myeloproliferative syndromes. In the first two groups, at least half of th...

    journal_title:Annals of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/BF01700275

    authors: Dührsen U,Hossfeld DK

    更新日期:1994-11-01 00:00:00

  • Radiotherapy and PGEMOX/GELOX regimen improved prognosis in elderly patients with early-stage extranodal NK/T-cell lymphoma.

    abstract::The optimal treatment strategy for elderly patients with natural killer/T-cell lymphoma (NKTCL) remains to be established. A total of 63 elderly patients with newly diagnosed NKTCL were retrospectively reviewed. Among the patients with stage I-II disease, 58.3 % received radiotherapy (RT) ± chemotherapy, and 41.7 % re...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1007/s00277-015-2395-y

    authors: Bi XW,Xia Y,Zhang WW,Sun P,Liu PP,Wang Y,Huang JJ,Jiang WQ,Li ZM

    更新日期:2015-09-01 00:00:00

  • Oral high-dose sucrosomial iron vs intravenous iron in sideropenic anemia patients intolerant/refractory to iron sulfate: a multicentric randomized study.

    abstract::Iron deficiency anemia is among the most frequent causes of disability. Intravenous iron is the quickest way to correct iron deficiency, bypassing the bottleneck of iron intestinal absorption, the only true mechanism of iron balance regulation in human body. Intravenous iron administration is suggested in patients who...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-020-04361-3

    authors: Giordano G,Napolitano M,Di Battista V,Lucchesi A

    更新日期:2020-12-02 00:00:00

  • Treatment of late-onset hemorrhagic cystitis after allogeneic hematopoietic stem cell transplantation: the role of corticosteroids.

    abstract::We aimed to evaluate the treatments, particularly the role of corticosteroids, in patients with late-onset hemorrhagic cystitis (LOHC) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). One hundred and sixty-three consecutive patients who underwent non-T-cell-depleted allo-HSCT and met the criterion...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-018-3290-0

    authors: Mo XD,Zhang XH,Xu LP,Wang Y,Yan CH,Chen H,Chen YH,Han W,Wang FR,Wang JZ,Liu KY,Huang XJ

    更新日期:2018-07-01 00:00:00

  • Prevalence of chronic idiopathic neutropenia of adults among an apparently healthy population living on the island of Crete.

    abstract::The aim of the present study was to investigate the prevalence of chronic idiopathic neutropenia of adults (CINA) among an apparently healthy population born and living on the island of Crete. The study was carried out with 778 subjects, 392 men aged 16-78 years (median 43 years) and 386 women aged 15-79 years (median...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s002770050518

    authors: Papadaki HA,Xylouri I,Coulocheri S,Kalmanti M,Kafatos A,Eliopoulos GD

    更新日期:1999-07-01 00:00:00

  • Myeloproliferative disorders: complications, survival and causes of death.

    abstract::This retrospective single-center study compared thromboembolic and hemorrhagic complications, survival and causes of death in a cohort of 102 consecutive patients with myeloproliferative disorders (MPD). We included 17 patients with essential thrombocythemia (ET), 59 with polycythemia vera (PV), and 26 with osteomyelo...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s002779900136

    authors: Brodmann S,Passweg JR,Gratwohl A,Tichelli A,Skoda RC

    更新日期:2000-06-01 00:00:00

  • Antiapoptotic effect of interleukin-2 (IL-2) in B-CLL cells with low and high affinity IL-2 receptors.

    abstract::Although B chronic lymphocytic leukemia (B-CLL) cells express the alpha chain of the interleukin-2 (IL-2) receptor CD25, little is known about the effect of IL-2 on apoptosis in B-CLL cells. We have shown previously that stimulation of B-CLL cells with a CpG-oligonucleotide induces IL-2 high affinity receptors. In our...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-010-0994-1

    authors: Decker T,Bogner C,Oelsner M,Peschel C,Ringshausen I

    更新日期:2010-11-01 00:00:00

  • Bortezomib, doxorubicin, and dexamethasone combination therapy followed by thalidomide and dexamethasone consolidation as a salvage treatment for relapsed or refractory multiple myeloma: analysis of efficacy and safety.

    abstract::We conducted a phase 2 study with bortezomib, doxorubicin, and dexamethasone (PAD) followed by thalidomide and dexamethasone (TD) in patients with relapsed multiple myeloma (MM). Forty patients were enrolled between November 2005 and October 2007, with follow-up continuing until January 2009. Efficacy could be assesse...

    journal_title:Annals of hematology

    pub_type: 杂志文章,多中心研究

    doi:10.1007/s00277-010-0943-z

    authors: Lee SS,Suh C,Kim BS,Chung J,Joo YD,Ryoo HM,Do YR,Jin JY,Kang HJ,Lee GW,Lee MH,Shim H,Kim K,Yoon SS,Bang SM,Kim HY,Lee JJ,Park J,Lee DS,Lee JH,Korean Multiple Myeloma Working Party.

    更新日期:2010-09-01 00:00:00

  • Acute porphyrias: a German monocentric study of the biochemical, molecular genetic, and clinical data of 62 families.

    abstract::In Germany, analyses of clinical and laboratory features of patients with acute porphyrias are only available for hereditary coproporphyria (HCP) but not with other acute porphyrias, acute intermittent porphyria (AIP) and variegate porphyria (VP). The aim of the study was to analyze a large cohort of patients with par...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1007/s00277-019-03831-7

    authors: Bronisch O,Stauch T,Haverkamp T,Beykirch MK,Petrides PE

    更新日期:2019-12-01 00:00:00

  • Monoclonal and biclonal gammopathy in two patients infected with Bartonella henselae.

    abstract::Two immunocompetent patients with cat-scratch disease due to infection with Bartonella henselae developed monoclonal and biclonal gammopathy. Neither patient had evidence of any other known cause of plasma cell dyscrasia, and antibiotic eradication of Bartonella henselae infection resulted in the prompt disappearance ...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-003-0675-4

    authors: Krause R,Auner HW,Daxböck F,Mulabecirovic A,Krejs GJ,Wenisch C,Reisinger EC

    更新日期:2003-07-01 00:00:00

  • Differential plasma proteome profiles of mild versus severe β-thalassemia/Hb E.

    abstract::The severity of thalassemia is currently classified based on clinical manifestations and multiple tests. In the present study, we performed a plasma proteome analysis to identify differentially expressed proteins compared between normal subjects and patients with mild and severe forms of β-thalassemia/hemoglobin E (Hb...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-012-1629-5

    authors: Hatairaktham S,Srisawat C,Siritanaratkul N,Chiangjong W,Fucharoen S,Thongboonkerd V,Kalpravidh RW

    更新日期:2013-03-01 00:00:00

  • Multiple myeloma in elderly patients-a Portuguese multicentric real-life study.

    abstract::Patients older than 75 years old with multiple myeloma (MM) have shorter survival and are usually treated differently from what features in clinical trials. In this study, the authors characterized the Portuguese population of MM patients above 75 years old, treated between 2009 and 2016. We compared the outcomes obta...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1007/s00277-019-03640-y

    authors: João C,Bergantim R,Neves M,Chacim S,Afonso C,Barradas J,Bernardo M,Coelho H,Esteves G,Fraga C,Geraldes C,Gonçalves C,Jorge A,Macedo A,Mendonça T,Moreira A,Roque A,Sarmento AB,Trigo F,Vitória H,Esteves S,Lúcio P

    更新日期:2019-07-01 00:00:00

  • Autologous stem cell transplantation in first remission is associated with better progression-free survival in multiple myeloma.

    abstract::Autologous stem cell transplant (ASCT) is standard consolidation therapy in management of multiple myeloma (MM) patients. We reviewed records of all consecutive MM patients who underwent ASCT with high-dose melphalan at our center from year 2002 to 2016. A total of 141 ASCT were conducted (90 males and 51 females) wit...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-018-3370-1

    authors: Aggarwal M,Agrawal N,Yadav N,Verma P,Ahmed R,Mehta P,Kapoor J,Bhurani D

    更新日期:2018-10-01 00:00:00

  • Outcome of treatment in adults with Philadelphia chromosome-positive and/or BCR-ABL--positive acute lymphoblastic leukemia-retrospective analysis of Polish Adult Leukemia Group (PALG).

    abstract::Patients with Philadelphia chromosome-positive (Ph+) and/or BCR-ABL+ acute lymphoblastic leukemia (ALL) have extremely poor prognoses. Most of these patients have additional, heterogenous karyotype abnormalities, the majority of which have uncertain clinical significance. In this study we analyzed the clinical charact...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-006-0099-z

    authors: Wrzesień-Kuś A,Robak T,Pluta A,Zwolińska M,Wawrzyniak E,Wierzbowska A,Skotnicki A,Jakubas B,Hołowiecki J,Nowak K,Kuliczkowski K,Mazur G,Haus O,Dmoszyńska A,Adamczyk-Cioch M,Jedrzejczak WW,Paluszewska M,Konopka L,Pałyn

    更新日期:2006-06-01 00:00:00

  • Treatment of primary mediastinal large B cell lymphoma with an alternating chemotherapy regimen based on high-dose methotrexate.

    abstract::Primary mediastinal large B cell lymphomas (MLCL) differ from other diffuse large cell lymphomas, leading to a description as a separate entity in the current World Health Organization classification. Dose intensification improves long-term results, but no standard therapy has been established so far. We investigated ...

    journal_title:Annals of hematology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1007/s00277-008-0625-2

    authors: Fietz T,Knauf WU,Hänel M,Franke A,Freund M,Thiel E,East German Study Group on Hematology and Oncology-OSHO.

    更新日期:2009-05-01 00:00:00

  • B cell-activating factor: its clinical significance in multiple myeloma patients.

    abstract::B cell-activating factor (BAFF) is a cytokine that plays a major role in the maintenance of normal B-cell development and homeostasis. It has been suggested that in multiple myeloma (MM) it might have regulatory effects on the proliferation and viability of malignant plasma cells. The aim of this study was to evaluate...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-012-1470-x

    authors: Fragioudaki M,Boula A,Tsirakis G,Psarakis F,Spanoudakis M,Papadakis IS,Pappa CA,Alexandrakis MG

    更新日期:2012-09-01 00:00:00

  • Successful alemtuzumab retreatment in progressive B-cell chronic lymphocytic leukemia: a multicenter survey in 30 patients.

    abstract::Although retreatment with alemtuzumab in relapsing B-cell chronic lymphocytic leukemia (CLL) may be beneficial, there has thus far been no thorough analysis available on this topic. Data were collected from 30 chemotherapy-pretreated patients with progressive CLL who had received alemtuzumab twice in consecutive, dist...

    journal_title:Annals of hematology

    pub_type: 杂志文章,多中心研究

    doi:10.1007/s00277-011-1192-5

    authors: Fiegl M,Falkner F,Steurer M,Zojer N,Hopfinger G,Haslbauer F,Winder G,Voskova D,Andel J,Lang A,Brychtova Y,Mayer J,Greil R,Gastl G,Austrian Collaborative Study Group on Alemtuzumab in Chronic Lymphocytic Leukemia.,Czech Leuk

    更新日期:2011-09-01 00:00:00

  • Inverse correlation of plasma leptin and soluble transferrin receptor levels in beta-thalassemia patients.

    abstract::The aim of the study was to investigate the association of leptin with hematological parameters in beta-thalassemia patients in Greece. We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 beta-thalassemia patients (21 transfusion dependent and ...

    journal_title:Annals of hematology

    pub_type: 杂志文章

    doi:10.1007/s00277-002-0499-7

    authors: Dedoussis GV,Kyrtsonis MC,Andrikopoulos NE,Voskaridou E,Loutradis A

    更新日期:2002-09-01 00:00:00