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).

Clinic of Pediatric Hemato Oncology Department of Women's and Children's Health University of Padova 46010 Padova Italy

Department for Hematology and Oncology University Children's Hospital University of Belgrade 11000 Belgrade Serbia

Department of Hematology and Oncology Hyogo Prefectural Kobe Children's Hospital Kobe 650 0047 Japan

Department of Pediatric Hematology and Oncology Charles University 2nd Medical School and University Hospital Motol 150 06 Prague Czech Republic

Department of Pediatric Hematology and Oncology St Anna Children's Hospital Medical University of Vienna 1090 Vienna Austria

Department of Pediatric Oncology Sahlgrenska University Hospital University of Gothenburg 413 45 Gothenburg Sweden

Department of Pediatrics and Adolescent Medicine Li Ka Shing Faculty of Medicine The University of Hong Kong Queen Mary Hospital Pokfulam Hong Kong

Division of Pediatric Hematology Oncology Istanbul University Oncology Institute 34452 İstanbul Turkey

Division of Pediatrics Hematology and Oncology University Medical Center Ljubljana SI 1000 Ljubljana Slovenia

Dmitry Rogachev National Medical Research Center of Pediatric Hematology Oncology and Immunology 117198 Moscow Russia

Erasmus Medical Center Sophia Children's Hospital 3015 CN Rotterdam The Netherlands

NHL BFM Study Center and Pediatric Hematology and Oncology University Hospital Muenster 48149 Muenster Germany

Pediatric Oncology Department Hospital Clínico Universitario de Valencia 46010 Valencia Spain

Princess Máxima Center for Pediatric Oncology 3584 CS Utrecht The Netherlands

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