1. CRLF2 and IKZF1 abnormalities in childhood hematological malignancies other than B-cell Acute Lymphoblastic Leukemia.
- Author
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Moreno-Lorenzana D, Juárez-Velázquez R, Reyes-León A, Martínez-Anaya D, Juárez-Villegas L, Zapata Tarrés M, López Santiago N, and Pérez-Vera P
- Subjects
- Humans, Child, Male, Female, Child, Preschool, Adolescent, Infant, Biomarkers, Tumor genetics, Ikaros Transcription Factor genetics, Receptors, Cytokine genetics, Hematologic Neoplasms genetics, Hematologic Neoplasms pathology, Gene Rearrangement
- Abstract
Rearrangements and overexpression of CRLF2 are hallmarks of poor outcomes in BCR::ABL1 -like B-ALL, and CRLF2 overexpression is a high-risk marker in T-ALL. However, CRLF2 alterations in pediatric hematologic malignancies other than B-ALL have not been reported. In this study, we analyzed the CRLF2 overexpression, rearrangements ( P2RY8::CRLF2 and IGH::CRLF2 ), activation (pSTAT5 and pERK), and the expression of dominant-negative IKZF1 isoforms (Ik6 and Ik8), implied in CRLF2 dysregulation, in 16 pediatric patients (AML, n = 9; T-ALL, n = 3; LBL, n = 2; HL, n = 1; cytopenia, n = 1). A high frequency of CRLF2 rearrangements and overexpression was found in the 16 patients: 28.6% (4/14) showed CRLF2 overexpression, 93.8% (15/16) were positive for CRLF2 total protein (cell-surface and/or cytoplasmic), while 62.5% (10/16) were positive for P2RY8::CRLF2 and 12.6% (2/16) for IGH::CRLF2 . In addition, 43.8% (7/16) expressed Ik6 and Ik8 isoforms. However, only a few patients were positive for the surrogate markers pSTAT5 (14.3%; 2/14) and pERK (21.4%; 3/14).
- Published
- 2024
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