EClinicalMedicine (Jun 2024)

Marginal zone lymphoma international prognostic index: a unifying prognostic index for marginal zone lymphomas requiring systemic treatmentResearch in context

  • Luca Arcaini,
  • Côme Bommier,
  • Juan Pablo Alderuccio,
  • Michele Merli,
  • Nicole Fabbri,
  • Maria Elena Nizzoli,
  • Matthew J. Maurer,
  • Vittoria Tarantino,
  • Simone Ferrero,
  • Sara Rattotti,
  • Annalisa Talami,
  • Roberta Murru,
  • Arushi Khurana,
  • Raphael Mwangi,
  • Marina Deodato,
  • Emanuele Cencini,
  • Francesca Re,
  • Carlo Visco,
  • Andrew L. Feldman,
  • Brian K. Link,
  • Marcia Torresan Delamain,
  • Michele Spina,
  • Ombretta Annibali,
  • Alessandro Pulsoni,
  • Andrés J.M. Ferreri,
  • Caterina Cecilia Stelitano,
  • Elsa Pennese,
  • Thomas M. Habermann,
  • Luigi Marcheselli,
  • Sunwoo Han,
  • Isildinha M. Reis,
  • Marco Paulli,
  • Izidore S. Lossos,
  • James R. Cerhan,
  • Stefano Luminari

Journal volume & issue
Vol. 72
p. 102592

Abstract

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Summary: Background: Marginal zone lymphomas (MZL), comprised of three unique but related subtypes, lack a unifying prognostic score applicable to all the patients in need for systemic chemotherapy and/or immunotherapy. Methods: Patients from the prospective NF10 study (NCT02904577) with newly diagnosed MZL and receiving frontline systemic therapy at diagnosis or after observation were used to train a prognostic model. The primary endpoint was progression-free survival (PFS) from start of treatment. The model was externally validated in a pooled analysis of two independent cohorts from the University of Iowa and Mayo Clinic Molecular Epidemiology Resource and the University of Miami. Findings: We identified 501 eligible patients. After multivariable modeling, lactate dehydrogenase (LDH) above upper normal limit, hemoglobin <12 g/dL, absolute lymphocyte count <1 × 109/L, platelets <100 × 109/L, and MZL subtype (nodal or disseminated) were independently associated with inferior PFS. The proposed MZL International Prognostic index (MZL-IPI) combined these 5 factors, and we defined low (LRG, 0 factors, 27%), intermediate (IRG, 1–2 factors, 57%) and high (HRG, 3+ factors, 16%) risk groups with 5-y PFS of 85%, 66%, and 37%, respectively (c-Harrell = 0.64). Compared to the LRG, the IRG (Hazard Ratio [HR] = 2.30, 95% CI 1.39–3.80) and HRG (HR = 5.41, 95% CI 3.12–9.38) had inferior PFS. Applying the MZL-IPI to the pooled US cohort (N = 353), 94 (27%), 192 (54%), and 67 (19%) patients were classified as LRG, IRG, and HRG, respectively, and the model was validated for PFS (log-rank test p = 0.0018; c-Harrell = 0.578, 95% CI 0.54–0.62). The MZL-IPI was also prognostic for OS in both the training and the external validation sets. Interpretation: MZL-IPI is a new prognostic score for use in all patients with MZL considered for systemic treatment. Funding: The MER was supported by P50 CA97274 and U01 CA195568.

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