B-Lymphoblastic Leukemia/Lymphoma, NOS (BLLNOS)

Overview

B-lymphoblastic leukemia/lymphoma, NOS (BLLNOS) is a subtype of B-cell precursor acute lymphoblastic leukemia/lymphoma that lacks a specific recurrent cytogenetic or molecular abnormality sufficient for a more specific WHO classification. It encompasses cases not classified into defined genetic subtypes (e.g., BCR-ABL1+, ETV6-RUNX1+, hyperdiploidy, hypodiploidy). BCR-ABL1-like (Ph-like) ALL is an important subset characterized by a gene expression signature resembling BCR-ABL1-positive ALL but lacking the BCR-ABL1 fusion itself.

Cohorts in the corpus

  • mixed_pipseq_2017 — PIPseq pediatric pan-cancer cohort (Columbia University Medical Center), which includes B-ALL/BLLNOS cases among 101 high-risk pediatric patients PMID:28007021.

Recurrent alterations

  • PIPseq cohort: BCR-ABL1-like RNA-seq signature identified in a 9-year-old girl with relapsed/refractory B-cell ALL; confirmatory RT-PCR identified a NUP214-ABL1 fusion; addition of dasatinib to third-line induction produced deep remission enabling curative bone-marrow transplant PMID:28007021.
  • PIPseq cohort: NT5C2 D407Y pharmacogenomic variant conferring resistance to nucleoside-analog therapy identified in relapsed BLL — informed salvage regimen choice PMID:28007021.
  • PIPseq cohort: FOXP1-ABL1 and SMARCC2-PDGFRB fusions identified as TKI targets in BLL cases PMID:28007021.
  • PIPseq cohort: JAK1 K1026E and STAT5B I704L mutations in T-BLL (T-ALL); SDHC G75D variant of uncertain significance returned in one BLL patient PMID:28007021.
  • Germline WES of 372 pediatric cancer patients included BCP-ALL (precursor B-cell lymphoblastic leukemia) cases; LP/PVs in CHEK2 (4 of 5 carriers) and TP53 (c.586C>T recurrent) were found in BCP-ALL patients; CHEK2 burden OR=9.6 (p=2.42×10⁻⁴) PMID:29489754

Subtypes

  • BCR-ABL1-like (Ph-like) ALL: activating kinase fusions (ABL1, PDGFRB, JAK2, CRLF2 rearrangements) that may be targetable with TKIs or JAK inhibitors.

Therapeutic landscape

  • BCR-ABL1-like BLL with kinase fusions (e.g., NUP214-ABL1, FOXP1-ABL1): TKI addition (dasatinib) to chemotherapy demonstrated deep remission in PIPseq cohort PMID:28007021.

Sources

  • PMID:28007021 — Oberg et al. PIPseq pediatric pan-cancer sequencing program (n=101).

This page was processed by crosslinker on 2026-05-14. - PMID:29489754

This page was processed by wiki-cli on 2026-05-15.