TY - JOUR
T1 - Pediatric Precursor B-Cell Lymphoblastic Malignancies
T2 - From Extramedullary to Medullary Involvement
AU - Kroeze, Emma
AU - Arias Padilla, Laura
AU - the EICNHL and the i-BFM Study
AU - Bakker, Max
AU - Boer, Judith M.
AU - Hagleitner, Melanie M.
AU - Burkhardt, Birgit
AU - Mori, Takeshi
AU - Attarbaschi, Andishe
AU - Verdú-Amorós, Jaime
AU - Pillon, Marta
AU - Anderzhanova, Liliya
AU - Kabíčková, Edita
AU - Chiang, Alan K.S.
AU - Kebudi, Rejin
AU - Mellgren, Karin
AU - Lazic, Jelena
AU - Jazbec, Janez
AU - Meijerink, Jules P.P.
AU - Beishuizen, Auke
AU - Loeffen, Jan L.C.
N1 - 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.
PY - 2022/8/12
Y1 - 2022/8/12
N2 - 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).
AB - 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).
UR - https://www.scopus.com/pages/publications/85137895590
U2 - 10.3390/cancers14163895
DO - 10.3390/cancers14163895
M3 - Article
C2 - 36010889
AN - SCOPUS:85137895590
SN - 2072-6694
VL - 14
JO - Cancers
JF - Cancers
IS - 16
M1 - 3895
ER -
