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Diffuse large B-cell lymphoma

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Updated 2024 BSH guidelines for the diagnosis and management of diffuse large B-cell lymphoma.

Background

Overview

Definition
DLBCL is a heterogeneous class of aggressive non-Hodgkin's lymphoma characterized by the rapid growth and accumulation of abnormal, large B cells.
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Pathophysiology
The pathophysiology of DLBCL is complex and heterogeneous, characterized by variations in gene expression profiles and genetic alterations that contribute to its development and progression. These genetic alterations can affect a wide range of cellular processes, including B cell differentiation, B cell receptor signaling, activation of the NF-κB pathway, apoptosis, and epigenetic regulation. Chromosomal translocations and gene rearrangements associated with DLBCL include translocation of band 3q27 resulting in BCL6 gene rearrangement, translocation t(14;18) resulting in BCL2 gene rearrangement, and MYC gene rearrangement.
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Epidemiology
The incidence of DLBCL in Europe is estimated at 2.79 per 100,000 person-years, increasing from 0.3 per 100,000 person-years at ages 35-39 to 26.6 per 100,000 persons-year at ages 80-84.
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Risk factors
Known risk factors for DLBCL include advanced age, family history of lymphoma, exposure to pesticides, fertilizers, or alkylating agents, and certain primary immunodeficiencies and genetic abnormalities.
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Disease course
Clinically, DLBCL often presents with lymphadenopathy and constitutional B symptoms, including fever, night sweats, fatigue, and weight loss.
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Prognosis and risk of recurrence
DLBCL is a high-grade, aggressive type of non-Hodgkin's lymphoma, but the prognosis can vary based on factors such as the stage of the disease at diagnosis, the patient's overall health, and the response to treatment.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of diffuse large B-cell lymphoma are prepared by our editorial team based on guidelines from the British Society for Haematology (BSH 2024,2020,2019,2016), the European Society of Medical Oncology (ESMO 2018,2016,2015), and the British HIV Association (BHIVA 2014).
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Classification and risk stratification

Staging: as per ESMO 2015 guidelines, use the Ann Arbor classification system for staging DLBCL.
A
Modified Ann Arbor classification for non-Hodgkin's lymphoma
Lymph node involvement
None
Single lymph node region
≥ 2 lymph node regions on the same side of the diaphragm
Regional lymph nodes of a single extralymphatic organ/site (with or without other lymph node regions on the same side of the diaphragm)
Lymph node regions on both sides of the diaphragm
Distant (nonregional) lymph nodes
Extralymphatic organ/site involvement
None
Single organ/site
Multifocal (disseminated) involvement of ≥ 1 organs/sites
Spleen involvement
No
Yes
Stage cannot be fully assessed
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  • Prognostic scores

Diagnostic investigations

History and physical examination: as per ESMO 2015 guidelines, perform a physical examination and assess performance status and B symptoms in patients with DLBCL.
B
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  • Imaging for staging

  • Laboratory evaluation

  • Cardiac evaluation

Diagnostic procedures

Biopsy and histopathology
As per BSH 2024 guidelines:
Perform excision biopsy to provide optimal material for diagnosis.
B
Consider performing needle-core biopsy when a surgical approach is either impractical or entails excessive risk or delay.
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  • Ancillary testing

  • Bone marrow biopsy

  • Lumbar puncture

Medical management

General principles: as per BSH 2024 guidelines, diagnose DLBCL in a reference hematopathology laboratory with access to a full range of phenotypic and molecular investigations.
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  • Induction and consolidation therapy (young patients, early/low-risk disease)

  • Induction and consolidation therapy (young patients, advanced/high-risk disease)

  • Induction and consolidation therapy (elderly/frail patients)

  • CNS prophylaxis

  • Febrile neutropenia prophylaxis

  • Infection prophylaxis

  • Management of osteoporosis

Specific circumstances

Patients with plasmablastic lymphoma: as per ESMO 2018 guidelines, obtain immunohistochemistry tests (for CD38, CD20, PAX5, CD138, EBER, CD30, MUM-1, Kappa-lambda, HHV8, and ALK) as part of diagnostic assessment in HIV-negative patients with suspected plasmablastic lymphoma.
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  • Patients with HIV-associated DLBCL

  • Patients with HBV infection

  • Patients with CNS DLBCL (diagnosis)

  • Patients with CNS DLBCL (baseline imaging)

  • Patients with CNS DLBCL (ophthalmological assessment)

  • Patients with CNS DLBCL (cognitive assessment)

  • Patients with CNS DLBCL (induction chemotherapy)

  • Patients with CNS DLBCL (consolidation therapy)

  • Patients with CNS DLBCL (management of intraocular lymphoma)

  • Patients with CNS DLBCL (assessment of treatment response)

  • Patients with CNS DLBCL (salvage therapy)

  • Patients with primary mediastinal DLBCL (mediastinal biopsy)

  • Patients with primary mediastinal DLBCL (bone marrow biopsy)

  • Patients with primary mediastinal DLBCL (baseline imaging)

  • Patients with primary mediastinal DLBCL (fertility counseling)

  • Patients with primary mediastinal DLBCL (pregnant patients)

  • Patients with primary mediastinal DLBCL (induction therapy)

  • Patients with primary mediastinal DLBCL (consolidation therapy)

  • Patients with primary mediastinal DLBCL (assessment of treatment response)

  • Patients with primary mediastinal DLBCL (salvage therapy)

  • Patients with primary extranodal DLBCL

  • Patients with testicular DLBCL

  • Patients with breast DLBCL

  • Patients with bone DLBCL

  • Patients with gastric DLBCL

  • Patients with cutaneous DLBCL

  • Patients with intravascular large B-cell lymphoma

Patient education

General counseling: as per BSH 2024 guidelines, discuss and explain all diagnoses and treatment plans with the patient and their family or carer if appropriate. Refer to additional sources of support.
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Follow-up and surveillance

Clinical follow-up: as per ESMO 2015 guidelines, elicit careful history and perform physical examination every 3 months for 1 year, every 6 months for 2 further years, and then once a year with attention to the development of secondary tumors or other long-term side-effects of chemotherapy.
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  • Laboratory follow-up

  • Imaging follow-up

  • Repeat biopsy