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International Prognostic Score for Nodular Lymphocyte-Predominant Hodgkin Lymphoma

  • GLOW Consortium
  • Stanford University
  • St. Jude Children Research Hospital
  • Provincial Health Services Authority
  • King Faisal Specialist Hospital and Research Centre
  • University of Texas MD Anderson Cancer Center
  • University of Oxford
  • Peter Maccallum Cancer Centre
  • University Health Network
  • The University of Chicago
  • Duke University
  • Beatson Oncology Centre
  • Dana-Farber Cancer Institute
  • University of Rochester
  • University of Pennsylvania
  • Yale University
  • Royal Hospital Oman
  • Tom Baker Cancer Centre
  • University of Turin
  • Roswell Park Cancer Institute
  • University of Copenhagen
  • Norfolk and Norwich University Hospitals NHS Foundation Trust
  • Newcastle upon Tyne Hospitals
  • Indiana University Bloomington
  • Phoenix Children's Hospital
  • Vanderbilt University
  • University Hospitals of Leicester NHS Trust
  • Guy's and St Thomas' NHS Foundation Trust
  • Manchester University NHS Foundation Trust
  • Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology
  • University of Florida
  • Mayo Clinic Florida
  • Johns Hopkins University
  • Canberra Health Services
  • Australian National University
  • Campania Region and Federico II University Hospital Naples (RandD and DISMET)
  • Institut Curie
  • Birmingham Women's and Children's NHS Foundation Trust
  • Austin Health
  • Monash University
  • The University of Sydney
  • Fiona Stanley Hospital
  • Sir Charles Gairdner Hospital
  • University of Western Australia
  • St. Vincent's Hospital Melbourne
  • University of Melbourne
  • St. Vincent's Hospital Sydney
  • University of New South Wales
  • University of Notre Dame Australia
  • Queensland Health
  • University of Queensland
  • Box Hill Hospital
  • Liverpool Hospital
  • Mayo Clinic Rochester, MN
  • University of Cologne

Research output: Contribution to journalArticlepeer-review

17 Scopus citations

Abstract

PURPOSENodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare cancer, and large international cooperative efforts are needed to evaluate the significance of clinical risk factors and immunoarchitectural patterns (IAPs) for all stages of pediatric and adult patients with NLPHL.METHODSThirty-eight institutions participated in the Global nLPHL One Working Group retrospective study of NLPHL cases from 1992 to 2021. We measured progression-free survival (PFS), overall survival (OS), transformation rate, and lymphoma-specific death rate. We performed uni- and multivariable (MVA) Cox regression stratified by management to select factors for the lymphocyte-predominant international prognostic score (LP-IPS) validated by five-fold cross-validation.RESULTSWe identified 2,243 patients with a median age of 37 years (IQR, 23-51). The median follow-up was 6.3 years (IQR, 3.4-10.8). Most had stage I to II (72.9%) and few B symptoms (9.9%) or splenic involvement (5.4%). IAP was scored for 916 (40.8%). Frontline management included chemotherapy alone (32.4%), combined modality therapy (30.5%), radiotherapy alone (24.0%), observation after excision (4.6%), rituximab alone (4.0%), active surveillance (3.4%), and rituximab and radiotherapy (1.1%). The PFS, OS, transformation, and lymphoma-specific death rates at 10 years were 70.8%, 91.6%, 4.8%, and 3.3%, respectively. On MVA, IAPs were not associated with PFS or OS, but IAP E had higher risk of transformation (hazard ratio [HR], 1.81; P <.05). We developed the LP-IPS with 1 point each for age ≥45 years, stage III-IV, hemoglobin <10.5 g/dL, and splenic involvement. Increasing LP-IPS was significantly associated with worse PFS (HR, 1.52) and OS (HR, 2.31) and increased risk of lymphoma-specific death (HR, 2.63) and transformation (HR, 1.41).CONCLUSIONIn this comprehensive study of all ages of patients with NLPHL, we develop the LP-IPS to identify high-risk patients and inform upcoming prospective clinical trials evaluating de-escalation of therapy for patients with low LP-IPS scores (<2).

Original languageEnglish
Pages (from-to)2271-2280
Number of pages10
JournalJournal of Clinical Oncology
Volume42
Issue number19
DOIs
StatePublished - Jul 1 2024

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