Lymphoblastic Lymphoma: a Concise Review

Curr Oncol Rep. 2022 Jan;24(1):1-12. doi: 10.1007/s11912-021-01168-x. Epub 2022 Jan 20.


Purpose of review: Lymphoblastic lymphoma (LBL) is a rare, highly aggressive non-Hodgkin lymphoma variant virtually indistinguishable from acute lymphoblastic leukemia (ALL). We review the advancements in diagnostics, staging, treatment, and response assessment.

Recent findings: T-LBL displays a mediastinal mass with pleuro-pericardic effusions as key distinctive features and is far more frequent than B-LBL. LBL is exquisitely sensitive to ALL-type chemotherapy, achieving cure rates in the order of 70% in adults and even more in children. Positron-emission tomography, genetic risk classifications, and minimal disseminated/residual disease assays are increasingly used to detect occult sites of involvement and predict treatment outcome. Stem cell transplantation is effective and should be considered for very high-risk subsets and/or at salvage. Although curable in the majority of patients, about 25-30% of adults with LBL patients experience resistance or relapse following first-line therapy. It is essential to identify these cases early on and to explore new modalities of precision medicine with targeted agents.

Keywords: Diagnosis; Future perspectives; Hematopoietic cell transplantation; Lymphoblastic lymphoma; Modern treatment; Risk factors; Staging.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Lymphoma, B-Cell*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / diagnosis
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / genetics
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma* / therapy
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / diagnosis
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / pathology
  • Precursor T-Cell Lymphoblastic Leukemia-Lymphoma* / therapy
  • Prognosis