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CD371-positive pediatric B-cell acute lymphoblastic leukemia: propensity to lineage switch and slow early response to treatment.
- Source :
-
Blood [Blood] 2024 Apr 25; Vol. 143 (17), pp. 1738-1751. - Publication Year :
- 2024
-
Abstract
- Abstract: In the effort to improve immunophenotyping and minimal residual disease (MRD) assessment in acute lymphoblastic leukemia (ALL), the international Berlin-Frankfurt-Münster (iBFM) Flow Network introduced the myelomonocytic marker CD371 for a large prospective characterization with a long follow-up. In the present study, we aimed to investigate the clinical and biological features of CD371-positive (CD371pos) pediatric B-cell precursor ALL (BCP-ALL). From June 2014 to February 2017, 1812 pediatric patients with newly diagnosed BCP-ALLs enrolled in trial AIEOP-BFM ALL 2009 were evaluated as part of either a screening (n = 843, Italian centers) or validation cohort (n = 969, other iBFM centers). Laboratory assessment at diagnosis consisted of morphological, immunophenotypic, and genetic analysis. Response assessment relied on morphology, multiparametric flow cytometry (MFC), and polymerase chain reaction (PCR)-MRD. At diagnosis, 160 of 1812 (8.8%) BCP-ALLs were CD371pos. This correlated with older age, lower ETV6::RUNX1 frequency, immunophenotypic immaturity (all P < .001), and strong expression of CD34 and of CD45 (P < .05). During induction therapy, CD371pos BCP-ALLs showed a transient myelomonocytic switch (mm-SW: up to 65.4% of samples at day 15) and an inferior response to chemotherapy (slow early response, P < .001). However, the 5-year event-free survival was 88.3%. Among 420 patients from the validation cohort, 27 of 28 (96.4%) cases positive for DUX4-fusions were CD371pos. In conclusion, in the largest pediatric cohort, CD371 is the most sensitive marker of transient mm-SW, whose recognition is essential for proper MFC MRD assessment. CD371pos is associated to poor early treatment response, although a good outcome can be reached after MRD-based ALL-related therapies.<br /> (© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.)
- Subjects :
- Humans
Child
Male
Female
Child, Preschool
Adolescent
Infant
Antineoplastic Combined Chemotherapy Protocols therapeutic use
Tetraspanins genetics
Tetraspanins metabolism
Immunophenotyping
Cell Lineage
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma genetics
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma mortality
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma drug therapy
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma pathology
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma diagnosis
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma therapy
Precursor B-Cell Lymphoblastic Leukemia-Lymphoma metabolism
Neoplasm, Residual diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1528-0020
- Volume :
- 143
- Issue :
- 17
- Database :
- MEDLINE
- Journal :
- Blood
- Publication Type :
- Academic Journal
- Accession number :
- 38215390
- Full Text :
- https://doi.org/10.1182/blood.2023021952