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Published on: December 28, 2015
Large B-cell lymphoma (LBCL): EHA Clinical Practice Guidelines for diagnosis, treatment, and follow-up
Catherine Thieblemont1,2, Maria Gomes Da Silva3, Sirpa Leppä4
1Université Paris Cité Paris France.
Insights
Large B-cell lymphoma (LBCL) diagnosis requires specialized labs and prognostic markers like MYC/BCL2 rearrangements. Treatment strategies, including R-CHOP and CAR T-cell therapy, are tailored to patient fitness and disease stage, addressing relapsed/refractory cases.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Large B-cell lymphoma (LBCL) constitutes a significant portion of adult lymphoma cases.
- Accurate diagnosis relies on hematopathology, immunophenotypic analysis, and molecular markers (MYC, BCL2).
- Prognostic tools like the International Prognostic Index (IPI) and imaging (PET-CT) are crucial for staging and response assessment.
Purpose of the Study:
- To outline current diagnostic and prognostic approaches for LBCL.
- To review first-line and subsequent treatment strategies for LBCL.
- To highlight management considerations for specific patient populations and relapsed/refractory disease.
Main Methods:
- Review of diagnostic criteria including immunophenotyping and molecular analysis.
- Analysis of staging and prognostic indicators such as PET-CT and IPI.
- Evaluation of treatment protocols including R-CHOP, Pola-R-CHP, and CAR T-cell therapy.
- Consideration of management for elderly, frail, and relapsed/refractory patients.
Main Results:
- LBCL diagnosis requires specialized pathology and molecular testing.
- PET-CT and IPI are vital for staging, prognosis, and treatment response.
- First-line treatments like R-CHOP/Pola-R-CHP are chosen based on IPI.
- CAR T-cell therapy and bispecific antibodies are key for relapsed/refractory LBCL.
Conclusions:
- LBCL management is multifaceted, involving precise diagnosis, risk stratification, and tailored therapies.
- Treatment selection depends on patient factors, disease stage, and response to prior therapies.
- Long-term follow-up is essential to manage treatment-related toxicities and late effects.
Abstract:
Large B-cell lymphoma (LBCL) accounts for about one-third of adult lymphoma cases. Diagnosis requires specialized hematopathology laboratories, with immunophenotypic analysis essential for confirming B-cell lineage and identifying variants. MYC and BCL2 rearrangements indicate a poor prognosis. Staging and prognosis rely on positron emission tomography computed tomography (PET-CT). The International Prognostic Index (IPI) aids risk stratification. PET-CT is critical for assessing treatment response and guiding strategies. First-line management for LBCL can be informed by interim PET to assess chemosensitivity, with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) or polatuzumab vedotin rituximab, cyclophosphamide, doxorubicin, and prednisone (Pola-R-CHP) for advanced stages depending on IPI scores. Primary mediastinal B-cell lymphoma (PMBCL) management favors R-CHOP given every 14 days (R-CHOP14) or dose-adjusted etoposide, doxorubicin, vincristine, cyclophosphamide, prednisone, and rituximab (DA-EPOCH-R) without radiotherapy in complete responders. Elderly patients, unfit or not (≥80 years or <80 with poor fitness), need geriatric assessment to guide therapy, often R-miniCHOP or non-anthracycline regimens. Frail patients should have adapted treatments. Prephase corticosteroids improve performance status, and supportive treatment should be optimized. The value of central nervous system (CNS) prophylaxis remains uncertain. CNS-IPI scores and specific anatomical sites help identify high-risk patients; magnetic resonance imaging (MRI) and colony-stimulating factor (CSF) analysis are recommended. Approximately 30%-40% of patients with LBCL experience relapsed or refractory disease after 1L treatment. Treatment strategies vary based on the timing of relapse (<1 year or ≥1 year). For those refractory or relapsing within <1 year and fit for therapy, chimeric antigen receptor T (CART) are the gold standard in 2L. CART in CART-naïve patients and bispecific antibodies appear to be the best approach in 3L. Follow-up includes clinical examination for 2 years and management for long-term side effects, such as cardiotoxicity, osteoporosis, immune dysfunction, neurocognitive impairment, endocrine dysfunction, fatigue, neuropathy, and mental distress.
