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

on behalf of the European Intergroup for Childhood Non-Hodgkin Lymphoma (EICNHL) and the International Berlin–Frankfurt–Münster (i-BFM) Study

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review

4 Citaten (Scopus)

Samenvatting

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).

Originele taal-2Engels
Artikelnummer3895
TijdschriftCancers
Volume14
Nummer van het tijdschrift16
DOI's
StatusGepubliceerd - 12 aug. 2022

Trefwoorden

  • B-cell acute lymphoblastic leukemia
  • B-cell lymphoblastic lymphoma
  • BCP-ALL
  • BCP-LBL
  • NHL
  • disease spectrum
  • non-Hodgkin lymphoma

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