How is the risk stratification for diffuse large cell lymphoma determined?

Updated: Jun 12, 2019
  • Author: Shipra Gandhi, MBBS; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Risk stratification plays an important role in the management of patients with diffuse large B-cell lymphoma (DLBCL) and should be performed before starting therapy.

The International Prognostic Index (IPI) score system was the result of a collaborative effort of 16 institutions in Europe and North America that used a dataset containing clinical information of almost 2000 patients. [20] Briefly, the IPI score system is calculated by the sum of the presence or absence of 5 variables easily available in most clinical practices (age ≥ 65 y, performance status ≥ 2, elevated lactate dehydrogenase (LDH), Ann Arbor stage III or IV, and ≥2 extranodal sites of disease). Based on the total score, DLBCL patients are assigned into 4 risk category groups (low, low-intermediate, high-intermediate, and high) with overall survival ranging from 23-75%.

The IPI score has been validated in multiple clinical trials before and after the incorporation of rituximab into the frontline therapy of patients with DLBCL. The IPI score has also been validated in relapsing aggressive non-Hodgkin lymphoma (NHL). [21]

In addition, modifications from the original predictive score have been formulated, such as the age-adjusted IPI score for patients younger than 65 years and the rituximab-IPI score with similar prognostic power.

While the clinical value of the IPI score is extremely important, especially when analyzing results across multiple clinical trials, it does not provide insightful information in regard to disease biology, including mechanisms of resistance to active treatments. This fact stresses the need to further identify and validate more biologically representative biomarkers of disease response using novel technology such as gene expression profiling (GEP), proteomics, or comparative chromosomal analysis.

In a large multi-center cohort, Alinari et al reported that patients with de novo CD5+ DLBCL have a poor prognosis despite initial rituximab-containing chemotherapy. Moreover, their results suggested that stem cell transplantation fails to salvage the majority of these patients [22] .


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