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  1. Article ; Online: COVID-19 infection and treatment with hydroxychloroquine cause severe haemolysis crisis in a patient with glucose-6-phosphate dehydrogenase deficiency.

    Beauverd, Yan / Adam, Yannick / Assouline, Benjamin / Samii, Kaveh

    European journal of haematology

    2020  Volume 105, Issue 3, Page(s) 357–359

    Abstract: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). ... ...

    Abstract Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). Hydroxychloroquine is suspected to trigger haemolytic crisis in G6PD-deficient patients, and off-label administration of this drug to patients infected with the novel coronavirus (SARS-CoV-2) could cause concern. We report here the first case of severe haemolytic crisis in a patient with G6PD deficiency, initiated by severe COVID-19 infection and hydroxychloroquine use. With worldwide spread of COVID-19, especially in regions with a high prevalence of G6PD deficiency, our case should alert physicians to this possible correlation.
    MeSH term(s) Aged ; Betacoronavirus ; COVID-19 ; Comorbidity ; Coronavirus Infections/complications ; Coronavirus Infections/drug therapy ; Coronavirus Infections/epidemiology ; Glucosephosphate Dehydrogenase Deficiency/complications ; Glucosephosphate Dehydrogenase Deficiency/epidemiology ; Hemolysis/drug effects ; Humans ; Hydroxychloroquine/adverse effects ; Hydroxychloroquine/therapeutic use ; Male ; Pandemics ; Pneumonia, Viral/drug therapy ; Pneumonia, Viral/epidemiology ; SARS-CoV-2
    Chemical Substances Hydroxychloroquine (4QWG6N8QKH)
    Keywords covid19
    Language English
    Publishing date 2020-05-07
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 392482-8
    ISSN 1600-0609 ; 0902-4441
    ISSN (online) 1600-0609
    ISSN 0902-4441
    DOI 10.1111/ejh.13432
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  2. Article ; Online: COVID‐19 infection and treatment with hydroxychloroquine cause severe haemolysis crisis in a patient with glucose‐6‐phosphate dehydrogenase deficiency

    Beauverd, Yan / Adam, Yannick / Assouline, Benjamin / Samii, Kaveh

    European Journal of Haematology

    2020  Volume 105, Issue 3, Page(s) 357–359

    Keywords Hematology ; General Medicine ; covid19
    Language English
    Publisher Wiley
    Publishing country us
    Document type Article ; Online
    ZDB-ID 392482-8
    ISSN 1600-0609 ; 0902-4441
    ISSN (online) 1600-0609
    ISSN 0902-4441
    DOI 10.1111/ejh.13432
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  3. Article: COVID-19 infection and treatment with hydroxychloroquine cause severe haemolysis crisis in a patient with glucose-6-phosphate dehydrogenase deficiency

    Beauverd, Yan / Adam, Yannick / Assouline, Benjamin / Samii, Kaveh

    Eur J Haematol

    Abstract: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). ... ...

    Abstract Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). Hydroxychloroquine is suspected to trigger haemolytic crisis in G6PD-deficient patients, and off-label administration of this drug to patients infected with the novel coronavirus (SARS-CoV-2) could cause concern. We report here the first case of severe haemolytic crisis in a patient with G6PD deficiency, initiated by severe COVID-19 infection and hydroxychloroquine use. With worldwide spread of COVID-19, especially in regions with a high prevalence of G6PD deficiency, our case should alert physicians to this possible correlation.
    Keywords covid19
    Publisher WHO
    Document type Article
    Note WHO #Covidence: #108769
    Database COVID19

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  4. Article ; Online: COVID-19 infection and treatment with hydroxychloroquine cause severe haemolysis crisis in a patient with glucose-6-phosphate dehydrogenase deficiency

    Beauverd, Yan / Adam, Yannick / Assouline, Benjamin / Samii, Kaveh

    ISSN: 0902-4441 ; European Journal of Haematology, Vol. 105, No

    2020  Volume 3, Issue pp. 357-359

    Abstract: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). ... ...

    Abstract Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an inherited genetic disorder caused by red cell enzymatic defects and is associated with haemolytic crisis when patients are exposed to oxidative agents (fava beans, drugs, infections). Hydroxychloroquine is suspected to trigger haemolytic crisis in G6PD-deficient patients, and off-label administration of this drug to patients infected with the novel coronavirus (SARS-CoV-2) could cause concern. We report here the first case of severe haemolytic crisis in a patient with G6PD deficiency, initiated by severe COVID-19 infection and hydroxychloroquine use. With worldwide spread of COVID-19, especially in regions with a high prevalence of G6PD deficiency, our case should alert physicians to this possible correlation.
    Keywords info:eu-repo/classification/ddc/616 ; info:eu-repo/classification/ddc/617 ; Coronavirus ; COVID-19 ; G6PD deficiency ; Haemolysis ; Hydroxychloroquine ; SARS-CoV-2 ; Aged ; Betacoronavirus ; Comorbidity ; Coronavirus Infections/complications/drug therapy/epidemiology ; Glucosephosphate Dehydrogenase Deficiency/complications/epidemiology ; Hemolysis/drug effects ; Humans ; Hydroxychloroquine/adverse effects/therapeutic use ; Male ; Pandemics ; Pneumonia ; Viral/drug therapy/epidemiology ; covid19
    Language English
    Publishing country ch
    Document type Article ; Online
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  5. Article ; Online: A Unique Case of Acquired Hemophilia A Presenting with Transient Ischemic Attack.

    Beauverd, Yan / Boehlen, Françoise / Fisch, Loraine / Sztajzel, Roman / Fontana, Pierre / Casini, Alessandro

    Acta haematologica

    2020  Volume 144, Issue 1, Page(s) 88–90

    Abstract: Acquired hemophilia A (AHA) is a rare but serious condition, usually associated with significant spontaneous or traumatic bleeding and a high mortality rate. In this report, we describe the case of an elderly patient presenting a transient ischemic ... ...

    Abstract Acquired hemophilia A (AHA) is a rare but serious condition, usually associated with significant spontaneous or traumatic bleeding and a high mortality rate. In this report, we describe the case of an elderly patient presenting a transient ischemic attack concurrently with AHA. A thrombotic event in AHA is occasionally associated with the use of bypassing agents for treatment, but a spontaneous thrombotic event has not ever been described.
    MeSH term(s) Aged, 80 and over ; Biomarkers ; Blood Coagulation ; Blood Coagulation Tests ; Diagnosis, Differential ; Factor VIII/administration & dosage ; Factor VIII/therapeutic use ; Hemophilia A/diagnosis ; Hemophilia A/etiology ; Hemophilia A/therapy ; Humans ; Ischemic Attack, Transient/diagnosis ; Ischemic Attack, Transient/etiology ; Ischemic Attack, Transient/therapy ; Male ; Recombinant Proteins/administration & dosage ; Recombinant Proteins/therapeutic use ; Symptom Assessment ; Tomography, X-Ray Computed ; Treatment Outcome
    Chemical Substances Biomarkers ; Recombinant Proteins ; Factor VIII (9001-27-8)
    Language English
    Publishing date 2020-03-10
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 80008-9
    ISSN 1421-9662 ; 0001-5792
    ISSN (online) 1421-9662
    ISSN 0001-5792
    DOI 10.1159/000506273
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  6. Article ; Online: Acute respiratory distress syndrome in a patient with primary myelofibrosis after ruxolitinib treatment discontinuation.

    Beauverd, Yan / Samii, Kaveh

    International journal of hematology

    2014  Volume 100, Issue 5, Page(s) 498–501

    Abstract: Ruxolitinib is a Janus kinase (JAK) inhibitor used for the treatment of myelofibrosis with demonstrated efficacy for the alleviation of disease-related symptoms and splenomegaly. Anemia and thrombocytopenia are the main secondary effects. However, there ... ...

    Abstract Ruxolitinib is a Janus kinase (JAK) inhibitor used for the treatment of myelofibrosis with demonstrated efficacy for the alleviation of disease-related symptoms and splenomegaly. Anemia and thrombocytopenia are the main secondary effects. However, there are case reports of rare but serious adverse events following drug withdrawal. We present a case of a 76-year-old man diagnosed with primary myelofibrosis who presented with constitutional symptoms and symptomatic splenomegaly. Ruxolitinib was started (15 mg twice daily) and his disease-related symptoms disappeared. Six weeks later, he developed grade 4 thrombocytopenia and grade 3 anemia. Ruxolitinib was stopped and corticosteroid treatment (prednisone 1 mg/kg/day) was started to avoid a cytokine-rebound reaction. The patient then developed fever, chills, a biological inflammatory syndrome, and an acute respiratory disease syndrome. Full workup excluded an infection and we concluded that ruxolitinib withdrawal syndrome was the likely cause. Continued treatment with corticosteroids, as well as oxygen supply and continuous positive airway pressure, allowed an alleviation of his symptoms. This case report describes acute respiratory distress syndrome as another potential complication of ruxolitinib withdrawal syndrome.
    MeSH term(s) Aged ; Anemia/chemically induced ; Humans ; Janus Kinases/antagonists & inhibitors ; Male ; Primary Myelofibrosis/complications ; Primary Myelofibrosis/diagnosis ; Primary Myelofibrosis/drug therapy ; Protein Kinase Inhibitors/adverse effects ; Protein Kinase Inhibitors/therapeutic use ; Pyrazoles/adverse effects ; Pyrazoles/therapeutic use ; Radiography, Thoracic ; Respiratory Distress Syndrome/diagnosis ; Respiratory Distress Syndrome/etiology ; Thrombocytopenia/chemically induced ; Tomography, X-Ray Computed
    Chemical Substances Protein Kinase Inhibitors ; Pyrazoles ; ruxolitinib (82S8X8XX8H) ; Janus Kinases (EC 2.7.10.2)
    Keywords covid19
    Language English
    Publishing date 2014-07-18
    Publishing country Japan
    Document type Case Reports ; Journal Article
    ZDB-ID 1076875-0
    ISSN 1865-3774 ; 0917-1258 ; 0925-5710
    ISSN (online) 1865-3774
    ISSN 0917-1258 ; 0925-5710
    DOI 10.1007/s12185-014-1628-5
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  7. Article ; Online: Complete remission of disseminated granulomatous dermatitis related to chronic eosinophilic leukemia following allogeneic stem cell transplantation.

    Beauverd, Yan / Cortés, Begonia / Masouye, Isabelle / Chalandon, Yves

    Leukemia & lymphoma

    2016  Volume 58, Issue 2, Page(s) 470–472

    MeSH term(s) Biopsy ; Bone Marrow/pathology ; Dermatitis/complications ; Dermatitis/pathology ; Dermatitis/therapy ; Female ; Granuloma/pathology ; Hematopoietic Stem Cell Transplantation/adverse effects ; Humans ; Hypereosinophilic Syndrome/diagnosis ; Hypereosinophilic Syndrome/etiology ; Leukemia/diagnosis ; Leukemia/etiology ; Leukocyte Count ; Middle Aged ; Neoplasms, Second Primary ; Remission Induction ; Skin/pathology ; Transplantation Conditioning/adverse effects ; Transplantation Conditioning/methods ; Transplantation, Homologous
    Language English
    Publishing date 2016-06-07
    Publishing country United States
    Document type Case Reports ; Letter
    ZDB-ID 1042374-6
    ISSN 1029-2403 ; 1042-8194
    ISSN (online) 1029-2403
    ISSN 1042-8194
    DOI 10.1080/10428194.2016.1190965
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  8. Article: Swiss Survey on current practices and opinions on clinical constellations triggering the search for PNH clones.

    Rovó, Alicia / Gavillet, Mathilde / Drexler, Beatrice / Keller, Peter / Schneider, Jenny Sarah / Colucci, Giuseppe / Beauverd, Yan / van Dorland, Hendrika Anette / Pollak, Matthias / Schmidt, Adrian / De Gottardi, Andrea / Bissig, Marina / Lehmann, Thomas / Duchosal, Michel A / Zeerleder, Sacha

    Frontiers in medicine

    2023  Volume 10, Page(s) 1200431

    Abstract: This national survey investigated the current practice in Switzerland by collecting participants' opinions on paroxysmal nocturnal hemoglobinuria (PNH) clone assessment and clinical practice.: Aim: This study aimed to investigate clinical indications ... ...

    Abstract This national survey investigated the current practice in Switzerland by collecting participants' opinions on paroxysmal nocturnal hemoglobinuria (PNH) clone assessment and clinical practice.
    Aim: This study aimed to investigate clinical indications prompting PNH clones' assessment and physician's accessibility of a flow cytometry facility, and also to understand clinical attitudes on the follow-up (FU) of patients with PNH clones.
    Methods: The survey includes 16 multiple-choice questions related to PNH and targets physicians with a definite level of experience in the topic using two screener questions. Opinion on clinical management was collected using hypothetical clinical situations. Each participant had the option of being contacted to further discuss the survey results. This was an online survey, and 264 physicians were contacted through email once a week for 5 weeks from September 2020.
    Results: In total, 64 physicians (24.2%) from 23 institutions participated (81.3% hematologists and 67.2% from university hospitals). All had access to flow cytometry for PNH clone testing, with 76.6% having access within their own institution. The main reasons to assess for PNH clones were unexplained thrombosis and/or hemolysis, and/or aplastic anemia (AA). Patients in FU for PNH clones were more likely to be aplastic anemia (AA) and symptomatic PNH. In total, 61% of the participants investigated PNH clones repetitively during FU in AA/myelodysplastic syndromes patients, even when there was no PNH clone found at diagnosis, and 75% of the participants tested at least once a year during FU. Opinions related to clinical management were scattered.
    Conclusion: The need to adhere to guidelines for the assessment, interpretation, and reporting of PNH clones emerges as the most important finding, as well as consensus for the management of less well-defined clinical situations. Even though there are several international guidelines, clear information addressing specific topics such as the type of anticoagulant to use and its duration, as well as the indication for treatment with complement inhibitors in some borderline situations are needed. The analysis and the discussion of this survey provide the basis for understanding the unmet needs of PNH clone assessment and clinical practice in Switzerland.
    Language English
    Publishing date 2023-07-26
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2775999-4
    ISSN 2296-858X
    ISSN 2296-858X
    DOI 10.3389/fmed.2023.1200431
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  9. Article ; Online: Low Plasma Levels of Hyaluronic Acid Might Rule Out Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation.

    De Ramón Ortiz, Carmen / Justo Sanz, Raul / Beauverd, Yan / Humala, Karem / López de la Guia, Ana / De Paz, Raquel / Gasior, Mercedes / Gómez Prieto, Pilar / Fabra Urdiola, Marta / Canales Albendea, Miguel / Butta, Nora / Jiménez Yuste, Victor

    Disease markers

    2023  Volume 2023, Page(s) 7589017

    Abstract: Background: Sinusoidal obstructive syndrome (SOS) is a potentially fatal complication secondary to hematopoietic stem cell transplant (HSCT) conditioning. Endothelial damage plasma biomarkers such as plasminogen activator inhibitor-1 (PAI-1), hyaluronic ...

    Abstract Background: Sinusoidal obstructive syndrome (SOS) is a potentially fatal complication secondary to hematopoietic stem cell transplant (HSCT) conditioning. Endothelial damage plasma biomarkers such as plasminogen activator inhibitor-1 (PAI-1), hyaluronic acid (HA), and vascular adhesion molecule-1 (VCAM1) represent potential diagnostic tools for SOS.
    Methods: We prospectively collected serial citrated blood samples (baseline, day 0, day 7, and day 14) in all adult patients undergoing HSCT at La Paz Hospital, Madrid. Samples were later analyzed by ELISA (enzyme-linked immunosorbent assay) for HA, VCAM1, and PAI-1 concentrations.
    Results: During sixteen months, we prospectively recruited 47 patients. Seven patients (14%) were diagnosed with SOS according to the EBMT criteria for SOS/VOD diagnosis and received treatment with defibrotide. Our study showed a statistically significant elevation of HA on day 7 in SOS patients, preceding clinical SOS diagnosis, with a sensitivity of 100%. Furthermore, we observed a significant increase of HA and VCAM1 levels on day 14. Regarding risk factors, we observed a statistically significant association between SOS diagnosis and the fact that patients received 3 or more previous lines of treatment before HSCT.
    Conclusions: The early significant increase in HA levels observed opens the door to a noninvasive peripheral blood test which could have the potential to improve diagnosis and facilitate prophylactic and therapeutic management of SOS before clinical/histological damage is established.
    MeSH term(s) Adult ; Humans ; Hepatic Veno-Occlusive Disease/diagnosis ; Hepatic Veno-Occlusive Disease/etiology ; Hepatic Veno-Occlusive Disease/drug therapy ; Hyaluronic Acid ; Plasminogen Activator Inhibitor 1 ; Polydeoxyribonucleotides/therapeutic use ; Hematopoietic Stem Cell Transplantation/adverse effects ; Vascular Cell Adhesion Molecule-1
    Chemical Substances Hyaluronic Acid (9004-61-9) ; Plasminogen Activator Inhibitor 1 ; Polydeoxyribonucleotides ; Vascular Cell Adhesion Molecule-1
    Language English
    Publishing date 2023-04-17
    Publishing country United States
    Document type Journal Article
    ZDB-ID 604951-5
    ISSN 1875-8630 ; 0278-0240
    ISSN (online) 1875-8630
    ISSN 0278-0240
    DOI 10.1155/2023/7589017
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  10. Article ; Online: Defibrotide Shows Efficacy in the Prevention of Sinusoidal Obstruction Syndrome After Allogeneic Hematopoietic Stem Cell Transplantation: A Retrospective Study.

    Chalandon, Yves / Mamez, Anne-Claire / Giannotti, Federica / Beauverd, Yan / Dantin, Carole / Mahne, Elif / Mappoura, Maria / Bernard, Fanette / de Ramon Ortiz, Carmen / Stephan, Caroline / Morin, Sarah / Ansari, Marc / Simonetta, Federico / Masouridi-Levrat, Stavroula

    Transplantation and cellular therapy

    2022  Volume 28, Issue 11, Page(s) 765.e1–765.e9

    Abstract: Sinusoidal obstruction syndrome (SOS), also known as hepatic veno-occlusive disease (VOD), is a well-known complication of allogeneic hematopoietic stem cell transplantation (HSCT) associated with a mortality rate of up to 85%.  Defibrotide has shown ... ...

    Abstract Sinusoidal obstruction syndrome (SOS), also known as hepatic veno-occlusive disease (VOD), is a well-known complication of allogeneic hematopoietic stem cell transplantation (HSCT) associated with a mortality rate of up to 85%.  Defibrotide has shown efficacy in treatment of SOS/VOD. Moreover, evidence exists supporting the efficacy of defibrotide as SOS/VOD prophylaxis. We have previously reported our single center experience on 52 HSCT recipients receiving defibrotide as SOS/VOD prophylaxis, which has shown that the patients did not develop any SOS/VOD under this prophylaxis. The aim of the present study was to see if we can confirm the previous results, mainly on the decrease incidence of SOS/VOD, as well as improve event-free survival (EFS) on a larger study population. We extended our previous study in a single-center retrospective analysis to include 237 consecutive patients (248 transplantations) who underwent transplantation between 1999 and 2009 for hematological diseases and receiving intravenous defibrotide as prophylaxis. This cohort was compared to 241 patients (248 transplantations) treated before 1999 or after 2009 when defibrotide prophylaxis was not routinely used in our center. Median follow-up for the study group was 10 (range 2-16) years and for the control group 2.7 (range 1-18) years. None of the 237 patients in the defibrotide group developed SOS/VOD. The cumulative incidence (CI) of SOS/VOD was 0% in the defibrotide group as compared to 4.8% (95% confidence interval [CI], 2.6-8%; P= .00046) in the control group. There was also a better 1-year EFS with 38% (95% CI, 32%-44%) in the defibrotide group versus 28% (95% CI, 22%-34%) (P= .00969) and decreased cumulative incidence of acute graft-versus-host disease (GvHD) in the defibrotide group 31% (95% CI, 25%-37%) versus 42% (95% CI, 36%-48%) (P= .026). The 1-year overall survival, relapse incidence, and non-relapse mortality were not statistically different. Multivariable analysis, performed taking into account clinical factors known to influence the risk of SOS/VOD, confirmed the favorable impact of defibrotide on SOS/VOD (HR 1.38e-08 [95% CI, 3.28e-09-5.80e-08]; P< .00001). Conversely, multivariable analysis failed to confirm the impact of defibrotide on 1-year EFS or acute GvHD. This large retrospective study on SOS/VOD-prophylaxis with defibrotide suggests that this approach may be of benefit. These results need to be confirmed in a prospective randomized trial.
    MeSH term(s) Humans ; Hepatic Veno-Occlusive Disease/drug therapy ; Retrospective Studies ; Prospective Studies ; Graft vs Host Disease/prevention & control ; Hematopoietic Stem Cell Transplantation/adverse effects
    Chemical Substances defibrotide (438HCF2X0M)
    Language English
    Publishing date 2022-08-08
    Publishing country United States
    Document type Journal Article
    ZDB-ID 3062231-1
    ISSN 2666-6367
    ISSN (online) 2666-6367
    DOI 10.1016/j.jtct.2022.08.003
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