| 1. |
- Kroeze, E., et al.
(författare)
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Pediatric Precursor B-Cell Lymphoblastic Malignancies: From Extramedullary to Medullary Involvement
- 2022
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Ingår i: Cancers. - : MDPI AG. - 2072-6694. ; 14:16
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Tidskriftsartikel (refereegranskat)abstract
- Simple Summary B-cell lymphoblastic lymphoma (BCP-LBL) and B-cell acute lymphoblastic leukemia (BCP-ALL) are both malignancies of immature B-cells. However, BCP-ALL has been extensively studied and treatment protocols have changed over the last decades, whereas BCP-LBL is quite rare, and treatment has stayed roughly the same. In this retrospective study, we compare the clinical characteristics of a cohort of BCP-LBL patients to a cohort BCP-ALL patients. With the comparison of this unique large cohort of immature B-cell malignancies, we aim to contribute to elucidating whether BCP-LBL and BCP-ALL represent two diseases, or different representations of the same disease. Increasing the understanding of BCP-LBL in comparison to BCP-ALL is crucial for improving treatment and prognosis for BCP-LBL. B-cell lymphoblastic lymphoma (BCP-LBL) and B-cell acute lymphoblastic leukemia (BCP-ALL) are the malignant counterparts of immature B-cells. BCP-ALL is the most common hematological malignancy in childhood, while BCP-LBL accounts for only 1% of all hematological malignancies in children. Therefore, BCP-ALL has been well studied and treatment protocols have changed over the last decades, whereas treatment for BCP-LBL has stayed roughly the same. Clinical characteristics of 364 pediatric patients with precursor B-cell malignancies were studied, consisting of BCP-LBL (n = 210) and BCP-ALL (n = 154) patients. Our results indicate that based on the clinical presentation of disease, B-cell malignancies probably represent a spectrum ranging from complete isolated medullary disease to apparent complete extramedullary disease. Hepatosplenomegaly and peripheral blood involvement are the most important discriminators, as both seen in 80% and 95% of the BCP-ALL patients and in 2% of the BCP-LBL patients, respectively. In addition, we show that the overall survival rates in this cohort differ significantly between BCP-LBL and BCP-ALL patients aged 1-18 years (p = 0.0080), and that the outcome for infants (0-1 years) with BCP-LBL is significantly decreased compared to BCP-LBL patients of all other pediatric ages (p < 0.0001).
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| 2. |
- Pritchard-Jones, K, et al.
(författare)
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The state of research into children with cancer across Europe : new policies for a new decade
- 2011
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Ingår i: ecancermedicalscience. - : Ecancer Global Foundation. - 1754-6605. ; 5, s. 210-
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Tidskriftsartikel (refereegranskat)abstract
- Overcoming childhood cancers is critically dependent on the state of research. Understanding how, with whom and what the research community is doing with childhood cancers is essential for ensuring the evidence-based policies at national and European level to support children, their families and researchers. As part of the European Union funded EUROCANCERCOMS project to study and integrate cancer communications across Europe, we have carried out new research into the state of research in childhood cancers. We are very grateful for all the support we have received from colleagues in the European paediatric oncology community, and in particular from Edel Fitzgerald and Samira Essiaf from the SIOP Europe office. This report and the evidence-based policies that arise from it come at a important junction for Europe and its Member States. They provide a timely reminder that research into childhood cancers is critical and needs sustainable long-term support.
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| 3. |
- Stark, D, et al.
(författare)
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Teenagers and young adults with cancer in Europe: from national programmes to a European integrated coordinated project.
- 2015
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Ingår i: European Journal of Cancer Care. - : Hindawi Limited. - 1365-2354 .- 0961-5423.
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Tidskriftsartikel (refereegranskat)abstract
- Over 14 000 patients aged 15-24 are estimated to be diagnosed with cancer in the European Union (EU) each year. Teenagers and young adults (TYA) often fall down gaps between children's and adults cancer services. The specific challenges of providing optimal care to them are described, but we present a summary of recent progress. Progress to overcome these challenges is happening at different rates across Europe. We summarise the European national projects in this field but more recently we have seen the beginnings of European coordination. Within the EU 7th Funding Programme (FP7) European Network for Cancer Research in Children and Adolescents programme (ENCCA), a specific European Network for Teenagers and Young Adults with Cancer has held a series of scientific meetings, including professionals, patients and caregivers. This group has proposed unanswered research questions and agreed key features of a high-quality service that can improve outcomes for TYA with cancer, including the primacy of collaboration between adult and paediatric services to eliminate the gap in the management of TYA with cancer.
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| 4. |
- Attarbaschi, A., et al.
(författare)
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Second malignant neoplasms after treatment of non-Hodgkin’s lymphoma—a retrospective multinational study of 189 children and adolescents
- 2021
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Ingår i: Leukemia. - : Springer Science and Business Media LLC. - 0887-6924 .- 1476-5551. ; 35, s. 534-549
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Tidskriftsartikel (refereegranskat)abstract
- Data on the spectrum of second malignant neoplasms (SMNs) after primary childhood non-Hodgkin’s lymphoma (NHL) are scarce. One-hundred-and-eighty-nine NHL patients diagnosed in a 30 years period of 1980–2010 developing an SMN were retrieved from 19 members of the European Intergroup for Childhood NHL and/or the international Berlin-Frankfurt-Münster Study Group. Five subgroups of SMNs were identified: (1) myeloid neoplasms (n = 43; 23%), (2) lymphoid neoplasms (n = 51; 27%), (3) carcinomas (n = 48; 25%), (4) central nervous system (CNS) tumors (n = 19; 10%), and (5) “other” SMNs (n = 28; 15%). In 37 patients (20%) preexisting disorders were reported with 90% having any kind of cancer predisposition syndrome (CPS). For the 189 primary NHL patients, 5-year overall survival (OS) after diagnosis of an SMN was 56 ± 4%, being worst for patients with preexisting disorders at 28 ± 8%. Five-year OS rates were 38 ± 8%, 59 ± 7%, 79 ± 8%, 34 ± 12%, and 62 ± 11%, respectively, for patients with myeloid and lymphoid neoplasms, carcinomas, CNS tumors, and “other” SMNs (p < 0.0001). Patients with SMNs after childhood NHL having a reported CPS, mostly mismatch repair disorders, carried a very poor prognosis. Moreover, although outcome was favorable in some subtypes of SMNs after childhood NHL (carcinomas, lymphoid neoplasms), other SMNs such as myeloid neoplasms and CNS tumors had a dismal prognosis. © 2020, The Author(s), under exclusive licence to Springer Nature Limited.
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| 5. |
- Meeths, M, et al.
(författare)
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Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) caused by deep intronic mutation and inversion in UNC13D
- 2011
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Ingår i: Blood. - Washington : American Society of Hematology. - 0006-4971 .- 1528-0020. ; 118:22, s. 5783-5793
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Tidskriftsartikel (refereegranskat)abstract
- Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive, often-fatal hyperinflammatory disorder. Mutations in PRF1, UNC13D, STX11, and STXBP2 are causative of FHL2, 3, 4, and 5, respectively. In a majority of suspected FHL patients from Northern Europe, sequencing of exons and splice sites of such genes required for lymphocyte cytotoxicity revealed no or only monoallelic UNC13D mutations. Here, in 21 patients, we describe 2 pathogenic, noncoding aberrations of UNC13D. The first is a point mutation localized in an evolutionarily conserved region of intron 1. This mutation selectively impairs UNC13D transcription in lymphocytes, abolishing Munc13-4 expression. The second is a 253-kb inversion straddling UNC13D, affecting the 3'-end of the transcript and likewise abolishing Munc13-4 expression. Carriership of the intron 1 mutation was found in patients across Europe, whereas carriership of the inversion was limited to Northern Europe. Notably, the latter aberration represents the first description of an autosomal recessive human disease caused by an inversion. These findings implicate an intronic sequence in cell-type specific expression of Munc13-4 and signify variations outside exons and splice sites as a common cause of FHL3. Based on these data, we propose a strategy for targeted sequencing of evolutionary conserved noncoding regions for the diagnosis of primary immunodeficiencies.
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| 6. |
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| 7. |
- Zuzak, Tycho J, et al.
(författare)
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Use of complementary and alternative medicine by children in Europe: Published data and expert perspectives
- 2013
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Ingår i: Complementary Therapies in Medicine. - : Elsevier. - 0965-2299 .- 1873-6963. ; 21, s. S34-S47
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Tidskriftsartikel (refereegranskat)abstract
- Introduction: Few data document the use of complementary and alternative medicine (CAM) in Europe, with even fewer investigating use by children. less thanbrgreater than less thanbrgreater thanMethods: A narrative, non-systematic review of CAM use in Europe was performed by combining data from published surveys with expert perspectives. Limitations created by a lack of representative studies, varying definitions of CAM use, and what qualifies as CAM in different countries was partially overcome by integrating local experts to summarise information available only in the national language and provide their perspectives about CAM availability, quality, use and popularity in their countries using a semi-structured questionnaire. Local and international published surveys were summarised, and the prevalence of CAM use was extrapolated. less thanbrgreater than less thanbrgreater thanResults: Data from 20 European countries were available, representing 69% of the European population. Some data about CAM use by the general population were available for 90% of the examined countries, whereas peer-reviewed published surveys were available for only 60%. We extrapolated that 56% (range: 10-90%, adjusted for population size) of the European population in general had used CAM at least once in the past year. Surveys in CAM use by children were available for 55% of the investigated countries. The extrapolated prevalence of CAM use by children in Europe was 52% (range: 5-90%, adjusted for population size). Paediatric CAM experts reported an increasing awareness for and use of CAM in healthcare institutions. less thanbrgreater than less thanbrgreater thanConclusion: This precursor for further surveys indicates that CAM appears to be popular not only among adults in Europe, but also for children. Development of a pan-European definition of CAM use and CAM therapies are required to achieve surveys comparable between European countries. Additionally, more research investigating the efficacy and potential adverse effects of CAM therapies is needed because of increasing CAM use by children in Europe.
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