Pediatric Precursor B-Cell Lymphoblastic Malignancies : From Extramedullary to Medullary Involvement
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).
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:14 |
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| Enthalten in: |
Cancers - 14(2022), 16 vom: 12. Aug. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Kroeze, Emma [VerfasserIn] |
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| Links: |
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| Themen: |
B-cell acute lymphoblastic leukemia |
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| Anmerkungen: |
Date Revised 08.03.2023 published: Electronic Citation Status PubMed-not-MEDLINE |
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| doi: |
10.3390/cancers14163895 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM345292839 |
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| 520 | |a 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) | ||
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| 700 | 1 | |a Boer, Judith M |e verfasserin |4 aut | |
| 700 | 1 | |a Hagleitner, Melanie M |e verfasserin |4 aut | |
| 700 | 1 | |a Burkhardt, Birgit |e verfasserin |4 aut | |
| 700 | 1 | |a Mori, Takeshi |e verfasserin |4 aut | |
| 700 | 1 | |a Attarbaschi, Andishe |e verfasserin |4 aut | |
| 700 | 1 | |a Verdú-Amorós, Jaime |e verfasserin |4 aut | |
| 700 | 1 | |a Pillon, Marta |e verfasserin |4 aut | |
| 700 | 1 | |a Anderzhanova, Liliya |e verfasserin |4 aut | |
| 700 | 1 | |a Kabíčková, Edita |e verfasserin |4 aut | |
| 700 | 1 | |a Chiang, Alan K S |e verfasserin |4 aut | |
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