PAGE CONTENTS

Prognosis

Diffuse large B-cell lymphoma (DLBCL) is the most common type of lymphoma, comprising around 25% to 30% of all cases of non-Hodgkin lymphoma (NHL). The disease usually presents with a rapidly growing mass or enlarged lymph nodes in nodal or extranodal sites.¹

For individuals with a recent diagnosis of DLBCL, there is considerable heterogeneity in survival.

To better stratify risk in patients and aid in the selection of therapeutic approaches, clinical scoring systems have been developed. The International Prognostic Index (IPI) scoring system has been extensively utilized since its initial publication over 25 years ago.²

The stage of the disease, histology, extranodal involvement, age, and performance status all affect the prognosis of any NHL. Imaging studies, laboratory values, and clinical examinations are used to monitor treatment response.¹

International Prognostic Index

The IPI is used to predict prognosis in DLBCL.¹ Negative prognostic factors correlated with reduced overall survival (OS) include:

age >60 years;
Eastern Cooperative Oncology Group (ECOG) performance status ≥2;
elevated levels of lactate dehydrogenase (LDH);
Ann Arbor clinical stage III/IV; and
>1 site with extranodal involvement.¹

The IPI score assigns 1 point to each of these negative prognostic factors and classifies patients into 4 risk groups based on the total score:

0 or 1 = low risk;
2 = low-intermediate risk;
3 = high-intermediate risk; and
4 or 5 = high risk.^2,3

Factors Affecting DLBCL Prognosis

Several variables influence prognosis, so it is impossible to provide a general statement on prognosis. However, DLBCL often responds favorably to treatment, and many individuals with this cancer are cured, meaning there is no longer any evidence of disease in the body following treatment. Factors that may impact prognosis include: age and overall health at the time of diagnosis; response to treatment; any genetic mutations; and the subtype of the disease.⁴

Unfavorable prognostic features include⁵:

Clinical features including: increasing age; elevated LDH levels; ECOG performance status ≥2; Ann Arbor stage III/IV; extranodal disease; and bulky disease
Cell of origin status: activated B-cell (ABC) subtype (40% of cases) and germinal center B-cell (GCB) subtype (60% of cases) show 5-year progression-free survival (PFS) rates of 40% to 50% and 70% to 80%, respectively
MYC translocation
Double expresser status: immunohistochemical coexpression of MYC in ≥40% of tumor cells and BCL2 in ≥50% tumor cells without coexistent MYC and BCL2 rearrangements
Bone marrow involvement is linked with a higher risk of central nervous system relapse

DLBCL Subtype and Prognosis

Up to 50% of individuals with DLBCL can be cured if they experience complete remission (CR) after receiving first-line therapy. The prognosis is better for the GCB subtype than the ABC subtype. Compared to DLBCL, double-hit and triple-hit lymphomas have a worse prognosis.¹

Read more about DLBCL types

DLBCL Survival Rate

Diffuse large B-cell lymphoma exhibits aggressive behavior, with a median OS of less than 1 year in untreated cases. The CHOP regimen, which consists of cyclophosphamide, hydroxydaunorubicin (sold as Lipodox® or Doxil®), Oncovin®, and prednisone, has been the mainstay of care since the 1970s, resulting in 50% of cases of CR and 30% to 40% of long survivors.⁶

A marked improvement in the prognosis of DLBCL has been seen in recent years, especially with the formation of R-CHOP. Rituxan® (rituximab), which was the first anti-CD20 monoclonal antibody to receive approval, was combined with the CHOP regimen of chemotherapy to become immunochemotherapy. Rituximab was added to first-line therapy, which increased CR by 15% to 20%. This increased 5-year event-free survival (EFS) from 29% to 47% in patients aged 60 to 80 years and 3-year EFS or PFS from 59% to 79% in patients aged 18 to 60 years. Immunochemotherapy considerably increased OS. As a result, by 2002, immunochemotherapy with rituximab became the new standard of treatment for DLBCL.⁶

Read more about DLBCL life expectancy

References

Padala SA, Kallam A. Diffuse large B cell lymphoma. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2022. Updated April 28, 2022. Accessed August 4, 2022.
Ruppert AS, Dixon JG, Salles G, et al. International prognostic indices in diffuse large B-cell lymphoma: a comparison of IPI, R-IPI, and NCCN-IPI. Blood. 2020;135(23):2041-2048. doi:10.1182/blood.2019002729
Survival rates and factors that affect prognosis (outlook) for non-Hodgkin lymphoma. American Cancer Society. Updated March 2, 2022. Accessed August 4, 2022.
Diffuse large B cell lymphoma (DLBCL). Lymphoma Australia. Accessed August 4, 2022.
Klairmont MM, Park CY. Lymphoma & related disorders: DLBCL, NOS. PathologyOutlines.com, Inc. Updated June 29, 2022. Accessed August 4, 2022.
Rovira J, Valera A, Colomo L, et al. Prognosis of patients with diffuse large B cell lymphoma not reaching complete response or relapsing after frontline chemotherapy or immunochemotherapy. Ann Hematol. 2015;94(5):803-812. doi:10.1007/s00277-014-2271-1

Reviewed by Hasan Avcu, MD, on 9/14/2022.

Harshi Dhingra, MD

Harshi Dhingra is a licensed medical doctor with specialization in Pathology. She is currently employed as faculty in a medical school with a tertiary care hospital and research center in India. Dr. Dhingra has over a decade of experience in diagnostic, clinical, research, and teaching work, and has written several publications and citations in indexed peer reviewed journals. She holds medical degrees for MBBS and an MD in Pathology.

Diffuse Large B-Cell Lymphoma (DLBCL)