CRLF2 rearrangement and extramedullary disease in B-cell acute lymphoblastic leukemia: a possible link?
Year: 2026
Type: article
Study design: Observational Study
Abstract: ABSTRACT: Cytokine receptor-like factor 2 rearrangement (CRLF2r), an aberration causing a Philadelphia chromosome-like gene expression profile and a poor prognostic marker in B-cell acute lymphoblastic leukemia (B-ALL), endows relative chemotherapeutic resistance. Extramedullary disease (EMD), including both central nervous system (CNS) and non-CNS involvement, can also be associated with worse outcomes. The relationship between CRLF2r and EMD is unknown. Based on an initial observation of patients with relapsed/refractory (R/R) CRLF2r B-ALL and EMD, a single-center retrospective review was conducted to evaluate the incidence of EMD in patients with CRLF2r B-ALL. Across 177 children, adolescents, and young adults with R/R B-ALL, 21 (11.9%) harbored CRLF2r, of whom 19 (90.5%) had EMD at some point during their treatment course. In contrast, only 78 of the 156 patients (50%) without CRLF2r ever had EMD (P = .0003). In the CRLF2r cohort, 8 (38.1%) presented with EMD at diagnosis: 7 with CNS and 1 with non-CNS EMD. At relapse, 17 (80.9%) had EMD: 4 (19%) had CNS EMD, 9 (42.9%) had non-CNS EMD, and 4 (19%) had both. Isolated EMD was observed in 2 patients (11.8%) at first relapse and in 3 (17.6%) with multiple relapses. Given these findings, further study to evaluate this potential association between EMD and CRLF2r in R/R B-ALL is indicated.
Source: Blood Advances
Institutions: National Institutes of Health, National Cancer Institute, Center for Cancer Research, Dana-Farber Cancer Institute, Johns Hopkins University, Johns Hopkins Hospital, National Heart, Lung, and Blood Institute, University of Pennsylvania, University of Wisconsin–Madison, University of Wisconsin Carbone Cancer Center, National Institutes of Health Clinical Center, Montefiore Medical Center, St. Jude Children's Research Hospital, Mayo Clinic
Language: English
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,800
Awards: G169511485, ECOG-ACRIN Network Group Operations Center, MINORITY-BASED COMMUNITY ONCOLOGY PROGRAM, G2490302200, G2554726104, G3100437649, G3475623468, National Clinical Trials Network Research at the University of Wisconsin, G9000082729, G9000280145, G9000327439, Development of Novel Approaches to Optimize CAR T-cell Therapies for B-cell Malignancies, Cancer Center Support Grant (CCSG)
OpenAlex ID: W7130340508