Kroeze Emma, Arias Padilla Laura, Bakker Max, Boer Judith M, Hagleitner Melanie M, Burkhardt Birgit, Mori Takeshi, Attarbaschi Andishe, Verdú-Amorós Jaime, Pillon Marta, Anderzhanova Liliya, Kabíčková Edita, Chiang Alan K S, Kebudi Rejin, Mellgren Karin, Lazic Jelena, Jazbec Janez, Meijerink Jules P P, Beishuizen Auke, Loeffen Jan L C
Princess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The Netherlands.
NHL-BFM Study Center and Pediatric Hematology and Oncology, University Hospital Muenster, 48149 Muenster, Germany.
Cancers (Basel). 2022 Aug 12;14(16):3895. doi: 10.3390/cancers14163895.
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).
B细胞淋巴母细胞淋巴瘤（BCP-LBL）和B细胞急性淋巴细胞白血病（BCP-ALL）是未成熟B细胞的恶性对应物。BCP-ALL是儿童期最常见的血液系统恶性肿瘤，而BCP-LBL仅占儿童所有血液系统恶性肿瘤的1%。因此，BCP-ALL已得到充分研究，在过去几十年中治疗方案发生了变化，而BCP-LBL的治疗大致保持不变。我们研究了364例前驱B细胞恶性肿瘤儿科患者的临床特征，其中包括BCP-LBL患者（n = 210）和BCP-ALL患者（n = 154）。我们的结果表明，根据疾病的临床表现，B细胞恶性肿瘤可能代表一个范围，从完全孤立的髓性疾病到明显的完全髓外疾病。肝脾肿大和外周血受累是最重要的鉴别因素，分别在80%的BCP-ALL患者和95%的BCP-ALL患者中出现，而在BCP-LBL患者中分别为2%。此外，我们表明，在该队列中，1至18岁的BCP-LBL和BCP-ALL患者的总生存率存在显著差异（p = 0.0080），并且与所有其他儿科年龄的BCP-LBL患者相比，0至1岁婴儿BCP-LBL的预后显著降低（p < 0.0001）。
