Lymphoma International Prognostic Index (IPI) Score

Predicts survival rate in patients with aggressive non-Hodgkin’s lymphoma.

In the text below the calculator there is more information about the two versions of the score, the original and the revised one.

The international prognostic index (IPI) score determines the survival rate at 4 and 5 years, via the original and the revised IPI scores.

This is a tool addressed to patients with aggressive non-Hodgkin’s lymphoma.

It is based on individual risk factors like patient age, cancer stage (Ann Arbor staging) or elevation in serum lactate dehydrogenase.

The outcome prognosis according to the international prognostic index (IPI) score is:

IPI score	Risk group	5-year survival	5-year survival according to PTCL*	3-year survival according to CDBCL*
0 - 1	Low	73%	91%	50%
2	Low - intermediate	51%	81%	33%
3	High - intermediate	43%	65%	16%
4 - 5	High	26%	59%	11%

The revised version of the score, the rIPI, provides the 4-year progression free survival for B-cell lymphoma:

Revised IPI score	Risk group	4-year progression free survival
0	Good	94%
1 – 2	Intermediate	80%
3 – 5	Poor	53%

Age greater than 60 years

Yes

Stage III or IV disease

Yes

Elevated serum LDH

Yes

ECOG performance status

0 - 1

3 - 4

Two or more extranodal sites of disease

Yes

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Steps on how to print your input & results:

1. Fill in the calculator/tool with your values and/or your answer choices and press Calculate.

2. Then you can click on the Print button to open a PDF in a separate window with the inputs and results. You can further save the PDF or print it.

Please note that once you have closed the PDF you need to click on the Calculate button before you try opening it again, otherwise the input and/or results may not appear in the pdf.

The lymphoma prognostic score explained

This prognosis score evaluates five risk factors in patients diagnosed with aggressive non-Hodgkin’s lymphoma. The final result is made out of the scores from each of the five items:

■ Age greater than 60 years;

■ Stage III or IV disease (according to the Ann Arbor* staging system);

■ Elevated serum lactate dehydrogenase;

■ ECOG performance status of 2 – 4;

■ Two or more extranodal sites of disease.

*The Ann Arbor classification is used to determine the stage of this disease but does not consistently distinguish between patients with different long-term prognoses.

All possible results and their survival rates are summarized in the following table:

IPI score	Risk group	5-year survival	5-year survival according to PTCL*	3-year survival according to CDBCL**
0 - 1	Low	73%	91%	50%
2	Low - intermediate	51%	81%	33%
3	High - intermediate	43%	65%	16%
4 - 5	High	26%	59%	11%

**Where PTCL refers to the International Peripheral T-cell Lymphoma Project and CDBCL refers to the CD20+ B-cell lymphoma study by Ziepert et al.

The main criticism of the model regards the fact that it may not be totally up to date as since it’s development, therapy for lymphoma has progressed significantly.

Rituximab therapy is now included in the standard therapeutic management of B-cell lymphomas and is likely to carry different survival prognosis than the doxorubicin-based chemotherapy regimens that were considered in the original study.

New therapy means have improved outcomes in lymphoma patients but their effect on IPI score has not been evaluated entirely.

In clinical trials, an age- adjusted IPI is often used. The score removes the item referring to age (on the assumption that the patients in the study are in the same age group) and the item referring to the presence of extranodal site factors.

The following table below introduces the correlation between the results from this adjusted (3-item IPI score) and predicted survival:

IPI score	Risk group	5-year survival
0	Low	83%
1	Low - intermediate	69%
2	High - intermediate	46%
3	High	32%

About the IPI study

The IPI score was created following the International Non-Hodgkin's Lymphoma Prognostic Factors Project in 1993, which reunited data from 2,031 patients with aggressive non-Hodgkin's lymphoma from 16 institutions and cooperative groups in the United States, Europe, and Canada.

The subjects have been treated with combination-chemotherapy regimens containing doxorubicin between 1982 and 1987.

The five risk factors found to have most relevant prediction value stratified patients in four risk groups, according to predicted five-year survival rates.

A separate group of 1,274 patients were analysed to determine the predictive capability of the age-adjusted model.

For both models, it was found that the increased risk of death was due to both a lower rate of complete responses and because of a higher rate of relapse from complete response.

The IPI was recommended for use in the design of future therapeutic trials in patients with aggressive non-Hodgkin's lymphoma and in disease therapeutic approaches.

Revised international prognostic index (rIPI)

The revised version of IPI is a valid predictor outcome in patients with aggressive B-cell lymphoma under therapy with a combination of rituximab and CHOP.

The following modifications were made:

In the original IPI, an ECOG result of 2 or greater was considered adverse outcome predictor and impacted on survival rate. In the revised IPI, ECOG scores of 3 or higher (instead of 2) are considered relevant for risk prediction.

There was a change of risk classification scale, the revised IPI operating with good, intermediate and poor whilst the original study operated with low, low-intermediate, high-intermediate and high.

The rIPI prognoses 4-year progression free survival, compared to the 5-year prediction in the IPI.

Revised IPI score	Risk group	4-year progression free survival
0	Good	94%
1 – 2	Intermediate	80%
3 – 5	Poor	53%

Other lymphoma conditions have separate survival stratification models, like the FLIPI score for follicular lymphoma or the MIPI score for mantle cell lymphoma.

About the rIPI study

The 2007 study by Sehn et al. was aimed at addressing the shortfalls of IPI and to update it in accordance to therapy progression. The addition of rituximab to CHOP chemotherapy (R-CHOP) has led to a significant improvement in the survival of patients with diffuse large B-cell lymphoma (DLBCL).

A retrospective analysis of patients with DLBCL treated with R-CHOP was done. It was found that although IPI remains predictive, it only identifies 2 risk groups.

The redistribution of the IPI factors created 3 risk groups and provided a clinically useful prediction of outcome.

Original source

1. International Non-Hodgkin's Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin's lymphoma. N Engl J Med. 1993; 329(14):987-94.

2. Sehn LH, Berry B, Chhanabhai M, Fitzgerald C, Gill K, Hoskins P, Klasa R, Savage KJ, Shenkier T, Sutherland J, Gascoyne RD, Connors JM. The revised International Prognostic Index (R-IPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R-CHOP. Blood. 2007; 109(5):1857-61.