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Pediatric Precursor B-Cell Lymphoblastic Malignancies: From Extramedullary to Medullary Involvement
E. Kroeze, L. Arias Padilla, M. Bakker, JM. Boer, MM. Hagleitner, B. Burkhardt, T. Mori, A. Attarbaschi, J. Verdú-Amorós, M. Pillon, L. Anderzhanova, E. Kabíčková, AKS. Chiang, R. Kebudi, K. Mellgren, J. Lazic, J. Jazbec, JPP. Meijerink, A....
Language English Country Switzerland
Document type Journal Article
Grant support
393
Foundation KiKa
NLK
Free Medical Journals
from 2009
PubMed Central
from 2009
Europe PubMed Central
from 2009
ProQuest Central
from 2009-01-01
Open Access Digital Library
from 2009-01-01
Open Access Digital Library
from 2009-01-01
ROAD: Directory of Open Access Scholarly Resources
from 2009
- Publication type
- Journal Article MeSH
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).
Department of Hematology and Oncology Hyogo Prefectural Kobe Children's Hospital Kobe 650 0047 Japan
Erasmus Medical Center Sophia Children's Hospital 3015 CN Rotterdam The Netherlands
Pediatric Oncology Department Hospital Clínico Universitario de Valencia 46010 Valencia Spain
Princess Máxima Center for Pediatric Oncology 3584 CS Utrecht The Netherlands
References provided by Crossref.org
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