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  • 1
    Language: English
    In: British journal of haematology, 2019-04, Vol.185 (2), p.360-363
    Subject(s): childhood leukaemia ; arsenic trioxide ; all‐trans retinoic acid ; acute promyelocytic leukaemia ; Hematologic Diseases - chemically induced ; Tretinoin - administration & dosage ; Humans ; Antineoplastic Combined Chemotherapy Protocols - adverse effects ; Child, Preschool ; Male ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Adolescent ; Tretinoin - adverse effects ; Female ; Arsenic Trioxide - adverse effects ; Arsenic Trioxide - administration & dosage ; Child ; Leukemia, Promyelocytic, Acute - drug therapy ; Arsenic ; Tretinoin ; 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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  • 2
    Language: English
    In: British journal of haematology, 2020-07, Vol.190 (1), p.93-104
    Description: Summary Diamond–Blackfan anaemia (DBA) is a rare and heterogeneous disease characterised by hypoplastic anaemia, congenital anomalies and a predisposition for malignancies. The aim of this paper is to report the findings from the Italian DBA Registry, and to discuss the Registry’s future challenges in tackling this disease. Our 20‐year long work allowed the connection of 50 Italian Association of Paediatric Haematology and Oncology (AIEOP) centres and the recruitment of 283 cases. Almost all patients have been characterised at a molecular level (96%, 271/283), finding a causative mutation in 68% (184/271). We confirm the importance of determination of erythrocyte adenosine deaminase activity (eADA) and of ribosomal RNA assay in the diagnostic pipeline and characterisation of a remission state. Patients with mutations in large ribosomal subunit protein (RPL) genes had a significant correlation with the incidence of malformations, higher eADA levels and more severe outcomes, compared to patients with mutations in small ribosomal subunit protein (RPS) genes. Furthermore, as a consequence of our findings, particularly the incidence of malignancies and the high percentage of patients aged 〉18 years, we stress the importance of collaboration with adult clinicians to guarantee regular multi‐specialist follow‐up. In conclusion, this study highlights the importance of national registries to increase our understanding and improve management of this complex disease.
    Subject(s): bone marrow failure ; erythrocyte adenosine deaminase ; RPS/RPL genes ; ribosomal RNA ; Diamond–Blackfan anaemia ; Humans ; Middle Aged ; Child, Preschool ; Male ; Young Adult ; Time Factors ; Anemia, Diamond-Blackfan - genetics ; Adolescent ; Adult ; Female ; Italy ; Registries ; Child ; 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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  • 3
    Language: English
    In: British journal of haematology, 2018-10, Vol.183 (2), p.298-301
    Description: Table SVI. Putative couples of miRNA and mRNA target with opposite correlated expression and predictive of relapse risk used for network construction.
    Subject(s): miRNA signature ; miRNA ; t(8;21)RUNX1‐RUNX1T1 ; relapse ; paediatric AML ; Gene expression ; Analysis ; MicroRNA ; 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: Pediatric blood & cancer, 2019-10, Vol.66 (10), p.e27892-n/a
    Description: Background Pediatric oncohematologic patients are a high‐risk population for clinical deterioration that might require pediatric intensive care unit (PICU) admission. Several studies have described outcomes and mortality predictors for patients post hematopoietic stem cell transplantation (HSCT), but fewer data exist regarding the category of non‐HSCT patients. Procedure All oncohematologic non‐HSCT patients ≤18 years requiring PICU admission from 1998 to 2015 in our tertiary‐care academic hospital were retrospectively evaluated by means of the pediatric hematology‐oncology unit database and the Italian PICUs data network database. We assessed the relation between demographic and clinical characteristics and 90‐day mortality after PICU admission. Results Of 3750 hospitalized oncohematologic patients, 3238 were non‐HSCT and 63 (2%) of them were admitted to the PICU. Patients were mainly affected by hematological malignancies (70%) and mostly were in the induction‐therapy phase. The main reasons for admission were respiratory failure (40%), sepsis (25%), and seizures (16%). The median PICU stay was 5 days (range 1‐107). The mortality rate at PICU discharge was 30%, and at 90 days it was 35%. Fifty‐five percent of deaths happened in the first 2 days of the PICU stay. Cardiac arrest (P = .007), presence of disseminated intravascular coagulation (DIC, P = .007), and acute kidney injury (AKI) at PICU admission (P 〈 .001) and during PICU stay (P = .021) were significant predictors of mortality in the multivariate analysis. Respiratory failure and mechanical ventilation were not associated with mortality. Conclusions A relatively small percentage of non‐HSCT patients required PICU admission, but the mortality rate was still high. Hemodynamic instability, DIC, and AKI, but not respiratory failure, were significant predictors of mortality.
    Subject(s): mortality ; prediction ; oncologic disease ; intensive care ; hematologic disease ; pediatric ; Intensive Care Units, Pediatric - statistics & numerical data ; Hematologic Neoplasms - mortality ; Humans ; Risk Factors ; Child, Preschool ; Female ; Infant ; Male ; Retrospective Studies ; Child ; Pediatric intensive care ; Patient outcomes ; Analysis ; Mortality ; Stem cells ; Transplantation ; Seizures (Medicine) ; Children ; Risk factors ; Hematopoietic stem cells ; Diseases ; Index Medicus
    ISSN: 1545-5009
    E-ISSN: 1545-5017
    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: Pharmaceutical research, 2016-02, Vol.33 (2), p.498-509
    Description: Here we show how a model-based approach may be used to provide further insight into the role of clinical and demographic covariates on the progression of iron overload. The therapeutic effect of deferoxamine is used to illustrate the application of disease modelling as a means to characterising treatment response in individual patients.Serum ferritin, demographic characteristics and individual treatment data from clinical routine practice on 27 patients affected by β-thalassaemia major were used for the purposes of this analysis. The time course of serum ferritin was described by a hierarchical nonlinear mixed effects model, in which compliance was parameterised as a covariate factor. Modelling and simulation procedures were implemented in NONMEM (7.2.0).A turnover model best described serum ferritin changes over time, with the effect of blood transfusions introduced on the ferritin conversion rate and the effect of deferoxamine on the elimination parameter (Kout) in a proportional manner. The results of the simulations showed that poor quality of execution is preferable over drug holidays; and that independently of the compliance pattern, the therapeutic intervention is not effective if 〉60% of the doses are missed.Modelling of ferritin response enables characterisation of the dynamics of iron overload due to chronic transfusion. The approach can be used to support decision making in clinical practice, including personalisation of the dose for existing and novel chelating agents.
    Subject(s): Biochemistry, general ; Biomedical Engineering ; iron overload ; deferoxamine ; Biomedicine ; disease modelling ; Pharmacy ; adherence ; dose rationale ; PKPD modelling ; Medical Law ; Pharmacology/Toxicology ; Deferoxamine - pharmacology ; beta-Thalassemia - therapy ; Erythrocyte Transfusion - adverse effects ; Iron Overload - drug therapy ; Humans ; Deferoxamine - therapeutic use ; beta-Thalassemia - complications ; Siderophores - pharmacology ; Iron Overload - blood ; Dose-Response Relationship, Drug ; Iron Overload - etiology ; Young Adult ; Ferritins - blood ; Models, Biological ; Computer Simulation ; Siderophores - therapeutic use ; Adolescent ; Siderophores - blood ; Adult ; Child ; Chelation Therapy - methods ; Deferoxamine - blood ; beta-Thalassemia - blood ; Models ; Blood transfusion ; Analysis ; Ferritin ; Index Medicus ; Research Paper
    ISSN: 0724-8741
    E-ISSN: 1573-904X
    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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  • 6
    Language: English
    In: Haematologica (Roma), 2018-01, Vol.103 (1), p.e1-e4
    Subject(s): Iron Chelating Agents - therapeutic use ; beta-Thalassemia - therapy ; Age Factors ; Iron Overload - drug therapy ; Humans ; Treatment Outcome ; beta-Thalassemia - complications ; beta-Thalassemia - diagnosis ; Iron Chelating Agents - adverse effects ; Iron Overload - etiology ; Deferiprone - adverse effects ; Deferiprone - administration & dosage ; Iron Chelating Agents - administration & dosage ; Mediterranean Region ; Deferiprone - therapeutic use ; Child ; Iron Overload - diagnosis ; Online Only
    ISSN: 0390-6078
    E-ISSN: 1592-8721
    Source: HighWire Press (Free Journals)
    Source: PubMed Central
    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: British journal of haematology, 2015-11, Vol.171 (4), p.566-573
    Description: Summary Paediatric patients with acute myeloid leukaemia (AML) who fail induction due to primary resistance to chemotherapy account for a significant proportion of cases and have a particularly dismal prognosis. We report the clinical and biological data, and final outcome of 48 paediatric patients with primary‐resistant AML enrolled in the Associazione Italiana di Ematologia e Oncologia Pediatrica AML 2002/01 clinical trial. These patients had a significantly higher white blood cell count at diagnosis compared to other AML patients. Cytogenetic and molecular features did not differ between patients with primary induction failure and patients allocated to the high‐risk group. For the whole patient population, the probability of overall survival, event‐free survival (EFS) and disease‐free survival (DFS) was 21·8% ± 6·2, 20·4% ± 5·9, and 49·5% ± 11·3, respectively. Twenty‐eight (58%) patients received haematopoietic stem cell transplantation (HSCT); 3 were autologous and 25 were allogeneic. Patients who underwent HSCT had improved EFS (31·2% vs. 5%, P 〈 0·0001). Only one of the 20 patients who did not receive HSCT is alive and disease free. The 19 patients in complete remission at time of HSCT showed significantly better DFS than the 9 with active disease (46% vs. 0%, P = 0·02). This study represents one of the largest series with long‐term follow up of paediatric AML patients with primary refractory disease. Children who underwent transplantation had an encouraging long‐term outcome. Disease recurrence remains the major cause of treatment failure; a better understanding of the disease biology is desirable to develop more effective treatment strategies.
    Subject(s): childhood leukaemia ; acute myeloid leukaemia ; relapse ; stem cell transplantation ; induction failure ; Recurrence ; Graft vs Host Disease - epidemiology ; Prognosis ; Follow-Up Studies ; Humans ; Child, Preschool ; Infant ; Male ; Transplantation, Autologous ; Incidence ; Allografts ; Treatment Failure ; Leukemia, Myeloid, Acute - drug therapy ; Female ; Child ; Infant, Newborn ; Leukemia, Myeloid, Acute - therapy ; Kaplan-Meier Estimate ; Hematopoietic Stem Cell Transplantation ; Remission Induction ; Leukemia, Myeloid, Acute - mortality ; Disease-Free Survival ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Adolescent ; Transplantation Conditioning ; Clinical trials ; Chemotherapy ; Transplantation ; Hematopoietic stem cells ; Cancer ; 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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  • 8
    Language: English
    In: Journal of pediatric hematology/oncology, 2020-08, Vol.42 (6), p.e472-e474
    Description: Diffuse large B-cell Lymphoma (DLBCL) secondary to a chronic severe Epstein-Barr virus (EBV) infection has not been previously described in a patient with trisomy 21. Here we report the case of a 14-year-old girl with trisomy 21 with impaired control of EBV and DLBCL. She was cured with dose-adapted chemotherapy and hematopoietic stem cell transplantation without severe treatment-related toxicity. We describe the first case of EBV-positive DLBCL in a patient with trisomy 21 and we propose a treatment modality for this rare entity.
    Subject(s): Prognosis ; Epstein-Barr Virus Infections - virology ; Humans ; Lymphoma, Large B-Cell, Diffuse - complications ; Combined Modality Therapy ; Down Syndrome - therapy ; Lymphoma, Large B-Cell, Diffuse - virology ; Herpesvirus 4, Human - isolation & purification ; Lymphoma, Large B-Cell, Diffuse - therapy ; Antineoplastic Combined Chemotherapy Protocols - therapeutic use ; Epstein-Barr Virus Infections - therapy ; Adolescent ; Down Syndrome - genetics ; Down Syndrome - complications ; Down Syndrome - virology ; Epstein-Barr Virus Infections - complications ; Female ; Hematopoietic Stem Cell Transplantation - methods ; Lymphoma, Large B-Cell, Diffuse - genetics ; Index Medicus
    ISSN: 1077-4114
    E-ISSN: 1536-3678
    Source: Hellenic Academic Libraries Link
    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: British journal of haematology, 2015-08, Vol.170 (3), p.398-407
    Description: Summary Imatinib mesylate (IM) is used for the management of childhood chronic myeloid leukaemia (CML). The most effective dosage of IM and its long‐term efficacy in children are not well defined. The purpose of this multicentre study is to report on the long‐term results of high‐dose IM (340 mg/m2/d) in CML patients in chronic phase (CP‐CML) aged 〈18 years at diagnosis. A total of 47 CP‐CML patients with a median age at diagnosis of 11 years 9 months were enrolled in nine Italian centres. Complete cytogenetic response was achieved in 91·5% of the evaluable patients at a median time of 6 months. BCR‐ABL1 International Scale ≤ 0·1% (major molecular response; MMR) and ≤0·01% (molecular response; MR) at 12 months were 66·6% and 33%, respectively. During follow‐up, MMR and MR were achieved in 78·6% and 61% of children, respectively. IM was safely discontinued in 3 long‐term treated children with a durable MR. Twelve patients (eight cytogenetic/molecular responders) underwent stem cell transplantation. The progression‐free survival probabilities at 96 months for responding patients who continued IM and for those transplanted were 60% and 50%, respectively. After a median follow‐up of 52 months (range 3–146), all patients are alive. High‐dose IM is a long‐term effective therapy in children and adolescents with CP‐CML.
    Subject(s): tyrosine kinase inhibitors ; imatinib ; BCR‐ABL1 ; childhood ; chronic myeloid leukaemia ; Piperazines - administration & dosage ; Follow-Up Studies ; Pyrimidines - administration & dosage ; Humans ; Child, Preschool ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - genetics ; Male ; Survival Rate ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - therapy ; Antineoplastic Agents - administration & dosage ; Imatinib Mesylate ; Stem Cell Transplantation ; Disease-Free Survival ; Benzamides - administration & dosage ; Fusion Proteins, bcr-abl - genetics ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - diagnosis ; Adolescent ; Female ; Italy ; Child ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - mortality ; Children ; 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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  • 10
    Language: English
    In: British journal of haematology, 2015-05, Vol.169 (4), p.584-589
    Description: Summary Sporadic essential thrombocythaemia (ET) is rare in paediatrics, and the diagnostic and clinical approach to paediatric cases cannot be simply copied from experience with adults. Here, we assessed 89 children with a clinical diagnosis of ET and found that 23 patients (25·8%) had a clonal disease. The JAK2 V617F mutation was identified in 14 children, 1 child had the MPL W515L mutation, and 6 had CALR mutations. The monoclonal X‐chromosome inactivation pattern was seen in six patients (two with JAK2 V617F and two with CALR mutations). The other 66 patients (74·2%) had persistent thrombocytosis with no clonality. There were no clinical or haematological differences between the clonal and non‐clonal patients. The relative proportion of ET‐specific mutations in the clonal children was much the same as in adults. The higher prevalence of non‐clonal cases suggests that some patients may not have myeloproliferative neoplasms, with significant implications for their treatment.
    Subject(s): CALR ; myeloproliferative neoplasm ; JAK2 ; essential thrombocythaemia ; paediatric ; Hematologic Neoplasms - therapy ; Humans ; Janus Kinase 2 - genetics ; Child, Preschool ; Infant ; Male ; Mutation, Missense ; Thrombocythemia, Essential - genetics ; Adolescent ; Thrombocythemia, Essential - therapy ; Adult ; Female ; Hematologic Neoplasms - genetics ; Neoplasm Proteins - genetics ; Child ; Amino Acid Substitution ; Cohort Studies ; Pediatrics ; Analysis ; Tumors ; 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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