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

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Kroeze E., Arias Padilla L., Bakker M., Boer J. M., Hagleitner M. M., Burkhardt B., ...Daha Fazla

Cancers, cilt.14, sa.16, 2022 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 14 Sayı: 16
  • Basım Tarihi: 2022
  • Doi Numarası: 10.3390/cancers14163895
  • Dergi Adı: Cancers
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, CAB Abstracts, CINAHL, EMBASE, Veterinary Science Database, Directory of Open Access Journals
  • Anahtar Kelimeler: 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
  • İstanbul Üniversitesi Adresli: Evet

Özet

© 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).