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  • 1
    Article
    Article
    2017
    ISSN: 0006-4971 
    Language: English
    In: Blood, 2017-05-25, Vol.129 (21), p.2824-2826
    Subject(s): Humans ; Immunosuppressive Agents ; Index Medicus ; Abridged Index Medicus
    ISSN: 0006-4971
    E-ISSN: 1528-0020
    Source: HighWire Press (Free Journals)
    Source: Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
    Source: American Society of Hematology
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 2
    Language: English
    In: Nature reviews. Immunology, 2020-06, Vol.20 (6), p.343-344
    Subject(s): Pandemics ; Pneumonia, Viral - therapy ; Coronavirus Infections - drug therapy ; Humans ; Complement System Proteins - immunology ; Respiratory Distress Syndrome - immunology ; Coronavirus Infections - immunology ; Pneumonia, Viral - immunology ; Drug Delivery Systems ; Betacoronavirus - immunology ; COVID-19 ; Respiratory Distress Syndrome - therapy ; SARS-CoV-2 ; Respiratory Distress Syndrome - virology ; Coronavirus Infections - therapy ; Immunotherapy ; Complement Inactivating Agents - therapeutic use ; Complement Activation - drug effects ; Medical colleges ; Health aspects ; Index Medicus
    ISSN: 1474-1733
    E-ISSN: 1474-1741
    Source: Nature Reviews
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 3
    Language: English
    In: British journal of haematology, 2018-09, Vol.182 (6), p.758-776
    Description: Summary This review examines the evidence that bone marrow failure (BMF) in aplastic anaemia (AA) is due to loss of haematopoietic stem cells (HSCs), which, in turn, is caused by deranged immunity and inflammation. We also consider how the course of the disease and the response to immuno‐suppressive therapy are influenced by the nature and specificity of the pathogenic process. A somatic mutation of the PIGA gene underlies the clonal disease paroxysmal nocturnal haemoglobinuria (PNH): there is direct evidence that the expansion of the PIGA mutant clone results from Darwinian selection exerted by a glycosyl‐phosphatidyl‐inositol ‐specific auto‐immune attack. Thus, PNH patients are a unique subset of patients with AA, in whom haematopoiesis recovers through this escape mechanism. A similar process, although less effective, may operate when the auto‐immune attack is against a human leucocyte antigen (HLA) molecule and an HLA mutation has produced a clone missing that molecule. We then discuss the significance of other mutant clones that are frequently found in AA, presumably due to a combination of genetic drift and selection. These clones are not causative of AA, but they emerge in AA and they may be pre‐leukaemic: unlike a PIGA mutant clone, in general they are unable to effectively reconstitute haematopoiesis.
    Subject(s): clonal expansion ; clonality ; PNH ; clonal evolution ; MDS ; aplastic anaemia ; Anemia, Aplastic - etiology ; Hematopoietic Stem Cells - immunology ; Animals ; Hematopoiesis ; Membrane Proteins - genetics ; Bone Marrow - pathology ; Humans ; Hematopoietic Stem Cells - pathology ; Hemoglobinuria, Paroxysmal ; Mutation ; Anemia, Aplastic - pathology ; Aplastic anemia ; Natural selection ; Index Medicus
    ISSN: 0007-1048
    E-ISSN: 1365-2141
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 4
    Language: English
    In: American journal of hematology, 2018-04, Vol.93 (4), p.564-577
    Description: Therapeutic complement inhibition by eculizumab has revolutionized the treatment of paroxysmal nocturnal hemoglobinuria (PNH) with a major impact on its natural history. Nevertheless, emerging unmet clinical needs may benefit from the development of novel complement inhibitors. Novel strategies of complement inhibition exploit different agents targeting C5, as well as compound intercepting the complement cascade at the level of its key component C3, or even upstream at the level of components involved in complement alternative pathway initiation. Many of these agents are already in their clinical development; preliminary data together with a deep understanding of PNH biology may help to anticipate their possible clinical effect. Novel anti‐C5 agents include monoclonal antibodies (even long‐lasting) as well as other small molecules bioavailable by subcutaneous administration; an anti‐C5 small interfering RNA has been developed too. All these anti‐C5 agents seem to recapitulate safety and efficacy of current eculizumab treatment; their main improvement pertains to better patient's convenience due to longer dosing interval and/or possible subcutaneous self‐administration. The possibility of achieving a deeper C5 inhibition has been shown as well, but its actual clinical meaning remains to be elucidated. Upstream complement inhibitors include the anti‐C3 small peptide compstatin (and its derivatives), and small inhibitors of complement factor D or complement factor B. This class of compounds anticipates a possible efficacy in prevention of C3‐mediated extravascular hemolysis, in addition to inhibition of intravascular hemolysis, eventually leading to improved hematological responses. The availability of all these compounds will result soon in a substantial improvement of PNH management.
    Subject(s): Antibodies, Monoclonal, Humanized - therapeutic use ; Drugs, Investigational - pharmacology ; Complement Inactivating Agents - pharmacology ; Humans ; Complement Inactivating Agents - therapeutic use ; Drug Design ; Hemoglobinuria, Paroxysmal - drug therapy ; Drugs, Investigational - therapeutic use ; Clinical Trials as Topic ; Complement Inactivating Agents - administration & dosage ; Forecasting ; Complement Activation - drug effects ; Monoclonal antibodies ; Hemolysis ; Complement inhibitors ; Complement component C3 ; Complement factor B ; Paroxysmal nocturnal hemoglobinuria ; siRNA ; Alternative pathway ; Complement factor D ; Index Medicus
    ISSN: 0361-8609
    E-ISSN: 1096-8652
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 5
    Language: English
    In: The New England journal of medicine, 2015-09-10, Vol.373 (11), p.1032-1039
    Description: Pregnancy has been discouraged in patients with paroxysmal nocturnal hemoglobinuria, a life-threatening hemolytic anemia, because of the heightened risk. However, in this study, eculizumab protected the mother and had no obvious adverse effect on the babies. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired stem-cell disorder that is characterized by chronic hemolysis, bone marrow failure, and venous thromboembolism. 1 – 3 Manifestations of the disease are related primarily to complement-mediated hemolysis. Patients with PNH present with a wide range of clinical symptoms, and if they do not receive specific treatment for the disorder, they can have a chronic, progressive illness and an increasing risk of death, primarily from thrombosis, over time (the median survival from the time of diagnosis ranges from 10 to 32 years). 1 – 3 Historically, the management of PNH during pregnancy has been challenging, and pregnancy . . .
    Subject(s): Antibodies, Monoclonal, Humanized - adverse effects ; Antibodies, Monoclonal, Humanized - therapeutic use ; Humans ; Premature Birth - epidemiology ; Pregnancy Complications, Hematologic - drug therapy ; Pregnancy ; Young Adult ; Fetal Death ; Adolescent ; Hemoglobinuria, Paroxysmal - drug therapy ; Adult ; Female ; Registries ; Surveys and Questionnaires ; Complement C5 - antagonists & inhibitors ; Monoclonal antibodies ; Care and treatment ; Dosage and administration ; Hemoglobinuria, Paroxysmal ; Mortality ; Analysis ; Postpartum period ; Fetuses ; Pregnancy complications ; Paroxysmal nocturnal hemoglobinuria ; Thrombosis ; Hemorrhage ; Quality of life ; Postpartum ; Complement activation ; Complement component C5 ; Womens health ; Children ; Heparin ; Thromboembolism ; Breast milk ; Index Medicus ; Abridged Index Medicus
    ISSN: 0028-4793
    E-ISSN: 1533-4406
    Source: Single Journals
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 6
    Language: English
    In: The New England journal of medicine, 2016-01-07, Vol.374 (1), p.43-53
    Description: Antilymphocyte globulin (ATG) added to the conditioning regimen before allogeneic hematopoietic stem-cell transplantation resulted in a lower rate of chronic graft-versus-host disease at 2 years than the rate without ATG (32% vs. 68%), with no apparent increased risk of relapse. Chronic graft-versus-host disease (GVHD) is a major complication of allogeneic stem-cell transplantation that results in later illness and death and a reduction in quality of life. 1 , 2 Risk factors for chronic GVHD are the use of peripheral blood as a source of stem cells, a history of acute GVHD, and the use of donated stem cells with high numbers of T cells. 3 – 7 In a meta-analysis, the Stem Cell Trialists’ Collaborative Group reported an incidence of extensive chronic GVHD of 47% after peripheral-blood stem-cell transplantation from an HLA-identical sibling. 4 In 2012, more than 70% of the stem-cell transplantations performed in . . .
    Subject(s): Graft vs Host Disease - epidemiology ; Prospective Studies ; Humans ; Immunosuppressive Agents - therapeutic use ; Middle Aged ; Proportional Hazards Models ; Child, Preschool ; Male ; Survival Rate ; Transplantation, Homologous ; Incidence ; Young Adult ; Disease-Free Survival ; Graft vs Host Disease - mortality ; Adolescent ; Antilymphocyte Serum - therapeutic use ; Adult ; Female ; Graft vs Host Disease - prevention & control ; T-Lymphocytes - immunology ; Child ; Chronic Disease ; Prevention ; Treatment outcome ; Graft versus host reaction ; Immunoglobulins ; Dosage and administration ; Analysis ; Graft-versus-host reaction ; Transplants & implants ; Leukemia ; Stem cell transplantation ; Lymphocytes T ; Preventive medicine ; Hemopoiesis ; Globulins ; Risk assessment ; Peripheral blood ; Stem cells ; Bone marrow ; Histocompatibility antigen HLA ; Index Medicus ; Abridged Index Medicus
    ISSN: 0028-4793
    E-ISSN: 1533-4406
    Source: Single Journals
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 7
    Language: English
    In: Digestive diseases and sciences, 2021-02, Vol.66 (2), p.408-411
    Description: Hepatitis-associated aplastic anemia is a well-recognized clinical syndrome in which marrow failure follows the development of hepatitis. Although aplastic anemia is intimately related to paroxysmal nocturnal hemoglobinuria, until now, no cases of PNH-associated hepatitis have been described. We report a case of recurrent acute hepatitis preceding the clinical onset of PNH. Treatment of PNH with the complement inhibitor eculizumab (Soliris ) prevented both recurrences of episodes of intravascular hemolysis and liver enzyme alteration. This is the first known published case of PNH-associated hepatitis.
    Subject(s): Hepatitis ; Enzymes ; Relapse ; Liver ; Aplastic anemia ; Development and progression ; Diseases ; Urine ; Thrombocytopenia ; Flow cytometry ; Dehydrogenases ; Leukopenia ; Urinalysis ; Anemia ; Infections ; Iron ; Patients ; Blood ; Proteins ; Ultrasonic imaging ; Granulocytes ; Biopsy ; Stem cells ; Bone marrow ; Epstein-Barr virus ; Viral infections ; Index Medicus ; Abridged Index Medicus
    ISSN: 0163-2116
    E-ISSN: 1573-2568
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 8
    Language: English
    In: American journal of hematology, 2016-04, Vol.91 (4), p.359-360
    Subject(s): Female ; Male ; Antibodies, Monoclonal, Humanized - therapeutic use ; Humans ; Hemoglobinuria, Paroxysmal - drug therapy ; Paroxysmal nocturnal hemoglobinuria ; Index Medicus
    ISSN: 0361-8609
    E-ISSN: 1096-8652
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 9
    Language: English
    In: Nature reviews. Immunology, 2020-07-01, Vol.20 (7), p.448-448
    Subject(s): Index Medicus
    ISSN: 1474-1733
    E-ISSN: 1474-1741
    Source: Nature Reviews
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 10
    Language: English
    In: The lancet oncology, 2015, Vol.16 (15), p.1525-1536
    Description: Summary Background The standard busulfan–cyclophosphamide myeloablative conditioning regimen is associated with substantial non-relapse mortality in patients older than 40 years with acute myeloid leukaemia who are undergoing allogeneic stem-cell transplantation. Because the combination of busulfan plus fludarabine has been proposed to reduce non-relapse mortality, we aimed to compare this treatment with busulfan plus cyclophosphamide as a preparative regimen in these patients. Methods We did an open-label, multicentre, randomised, phase 3 trial for patients with acute myeloid leukaemia at 25 hospital transplant centres in Italy and one in Israel. Eligible patients were aged 40–65 years, had an Eastern Cooperative Oncology Group performance status less than 3, and were in complete remission. Patients were randomly assigned 1:1 to receive intravenous busulfan plus cyclophosphamide or busulfan plus fludarabine. Treatment allocations were not masked to investigators or patients. Randomisation was done centrally via a dedicated web-based system using remote data entry, with patients stratified by donor type and complete remission status. Patients allocated to busulfan plus cyclophosphamide received intravenous busulfan 0·8 mg/kg four times per day during 2 h infusions for four consecutive days (16 doses from days −9 through −6; total dose 12·8 mg/kg) and cyclophosphamide at 60 mg/kg per day for two consecutive days (on days −4 and −3; total dose 120 mg/kg). Patients allocated to busulfan plus fludarabine received the same dose of intravenous busulfan (from days −6 through −3) and fludarabine at 40 mg/m2 per day for four consecutive days (from days −6 through −3; total dose 160 mg/m2 ). The primary endpoint was 1-year non-relapse mortality, which was assessed on an intention-to-treat basis; safety outcomes were assessed in the per-protocol population. This trial has been completed and is registered with ClinicalTrials.gov , number NCT01191957. Findings Between Jan 3, 2008, and Dec 20, 2012, we enrolled and randomly assigned 252 patients to receive busulfan plus cyclophosphamide (n=125) or busulfan plus fludarabine (n=127). Median follow-up was 27·5 months (IQR 9·8–44·3). 1-year non-relapse mortality was 17·2% (95% CI 11·6–25·4) in the busulfan plus cyclophosphamide group and 7·9% (4·3–14·3) in the busulfan plus fludarabine group (Gray's test p=0·026). The most frequently reported grade 3 or higher adverse events were gastrointestinal events (28 [23%] of 121 patients in the busulfan plus cyclophosphamide group and 26 [21%] of 124 patients in the busulfan plus fludarabine group) and infections (21 [17%] patients in the busulfan plus cyclophosphamide group and 13 [10%] patients in the busulfan plus fludarabine group had at least one such event). Interpretation In older patients with acute myeloid leukaemia, the myeloablative busulfan plus fludarabine conditioning regimen is associated with lower transplant-related mortality than busulfan plus cyclophosphamide, but retains potent antileukaemic activity. Accordingly, this regimen should be regarded as standard of care during the planning of allogeneic transplants for such patients. Funding Agenzia Italiana del Farmaco.
    Subject(s): Hematology, Oncology and Palliative Medicine ; Cyclophosphamide - administration & dosage ; Humans ; Middle Aged ; Hematopoietic Stem Cell Transplantation ; Induction Chemotherapy ; Male ; Vidarabine - analogs & derivatives ; Antineoplastic Agents - administration & dosage ; Busulfan - administration & dosage ; Transplantation, Homologous ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Leukemia, Myeloid, Acute - drug therapy ; Adult ; Female ; Leukemia, Myeloid, Acute - surgery ; Aged ; Vidarabine - administration & dosage ; Transplantation Conditioning ; Cyclophosphamide ; Product development ; Transplantation ; Busulfan ; Mortality ; Stem cells ; Index Medicus
    ISSN: 1470-2045
    E-ISSN: 1474-5488
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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