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Pediatric Precursor...
Pediatric Precursor B-Cell Lymphoblastic Malignancies: From Extramedullary to Medullary Involvement
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Kroeze, E. (author)
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Padilla, L. A. (author)
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Bakker, M. (author)
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Boer, J. M. (author)
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Hagleitner, M. M. (author)
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Burkhardt, B. (author)
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Mori, T. (author)
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Attarbaschi, A. (author)
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Verdu-Amoros, J. (author)
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Pillon, M. (author)
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Anderzhanova, L. (author)
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Kabickova, E. (author)
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Chiang, A. K. S. (author)
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Kebudi, R. (author)
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- Mellgren, Karin, 1962 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för kliniska vetenskaper, Avdelningen för pediatrik,Institute of Clinical Sciences, Department of Pediatrics
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Lazic, J. (author)
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Jazbec, J. (author)
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Meijerink, J. P. P. (author)
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Beishuizen, A. (author)
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Loeffen, J. L. C. (author)
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European Intergrp, Childhood (author)
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Int Berlin Frankfurt, Munster (author)
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(creator_code:org_t)
- 2022-08-12
- 2022
- English.
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In: Cancers. - : MDPI AG. - 2072-6694. ; 14:16
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Abstract
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- 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).
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
Keyword
- B-cell lymphoblastic lymphoma
- BCP-LBL
- B-cell acute lymphoblastic
- leukemia
- BCP-ALL
- non-Hodgkin lymphoma
- NHL
- disease spectrum
- leukemia
- children
- adolescents
- infants
- disease
- reduction
- lymphomas
- survival
- therapy
- trial
- Oncology
Publication and Content Type
- ref (subject category)
- art (subject category)
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Cancers
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- By the author/editor
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Kroeze, E.
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Padilla, L. A.
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Bakker, M.
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Boer, J. M.
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Hagleitner, M. M ...
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Burkhardt, B.
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show more...
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Mori, T.
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Attarbaschi, A.
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Verdu-Amoros, J.
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Pillon, M.
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Anderzhanova, L.
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Kabickova, E.
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Chiang, A. K. S.
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Kebudi, R.
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Mellgren, Karin, ...
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Lazic, J.
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Jazbec, J.
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Meijerink, J. P. ...
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Beishuizen, A.
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Loeffen, J. L. C ...
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European Intergr ...
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Int Berlin Frank ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Cancer and Oncol ...
- Articles in the publication
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Cancers
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University of Gothenburg