Pediatric Precursor B-Cell Lymphoblastic Malignancies: From Extramedullary to Medullary Involvement
Tarih
2022Yazar
Loeffen, Jan L. C.
Pillon, Marta
Anderzhanova, Liliya
Kabíčková, Edita
Chiang, Alan K. S.
Mellgren, Karin
Lazic, Jelena
Jazbec, Janez
Meijerink, Jules P. P.
Beishuizen, Auke
Kebudi, Rejin
Kroeze, Emma
Arias Padilla, Laura
Bakker, Max
Boer, Judith M.
Hagleitner, Melanie M.
Burkhardt, Birgit
Mori, Takeshi
Attarbaschi, Andishe
Verdú-Amorós, Jaime
Üst veri
Tüm öğe kaydını gösterÖzet
© 2022 by the authors.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).
Bağlantı
http://hdl.handle.net/20.500.12627/187025https://doi.org/10.3390/cancers14163895
https://avesis.istanbul.edu.tr/api/publication/234db765-35b9-49f2-988d-23757f8ece22/file
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