Pediatric Precursor B-Cell Lymphoblastic Malignancies: From Extramedullary to Medullary Involvement

Emma Kroeze, Laura Arias Padilla, on behalf of the EICNHL and the i-BFM Study, Max Bakker, Judith M. Boer, Melanie M. Hagleitner, Birgit Burkhardt, Takeshi Mori, Andishe Attarbaschi, Jaime Verdú-Amorós, Marta Pillon, Liliya Anderzhanova, Edita Kabíčková, Alan K.S. Chiang, Rejin Kebudi, Karin Mellgren, Jelena Lazic, Janez Jazbec, Jules P.P. Meijerink, Auke BeishuizenJan L.C. Loeffen*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

7 Citations (Scopus)
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Abstract

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).

Original languageEnglish
Article number3895
JournalCancers
Volume14
Issue number16
DOIs
Publication statusPublished - 12 Aug 2022

Bibliographical note

Funding: This research was funded by Kinderen Kankervrij, grant number 393 and Deutsche
Kinderkrebsstiftung (NHL-BFM Registry 2012, DKS 2014.11 and DKS 2016.24).

Publisher Copyright: © 2022 by the authors.

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