International prognostic score for asymptomatic early-stage CLL

Estimate likelihood of treatment requirement in patients with early-stage chronic lymphocytic leukemia


The IPS-E allows more individualized management of CLL patients in clinical practice. The IPS-E uses three variables (unmutated IGHV, lymphocytes >15,000/microL, palpable lymph nodes) to stratify patients with early stage CLL at diagnosis into three risk groups with differing likelihood of requiring treatment at one and five years.  Low-risk patients overall correspond to 29.5% of Binet stage A CLL. Their cumulative risk for treatment need is less than 0.1% after 1 year of surveillance, and 8.4% after 5 years. The risk for treatment is 2.0 events per 100 person years among low-risk patients. Intermediate-risk patients overall correspond to 36.2% of Binet stage A CLL. Their cumulative risk for treatment need is 3.1% after 1 year of surveillance, and 28.4% after 5 years. The need for therapy is 6.1 events per 100 person years among intermediate-risk patients. High-risk patients overall correspond to 34.3% of Binet stage A CLL. Their cumulative risk for treatment need is 14.1% after 1 year of surveillance and 61.2% after 5 years. The risk for treatment is 16.1 events per 100 person years among high-risk patients.  IPS-E high-risk patients should be followed more closely than low- and intermediate-risk patients because of the likelihood of requiring treatment sooner.

The IPS-E was developed and validated using individual patient data from 11 international cohorts of approximately 5000 patients with early stage CLL (Rai stage 0, I, or II or Binet stage A) initially managed with active surveillance. Unmutated IGHV, lymphocytes >15,000/microL, and palpable lymph nodes consistently and independently correlated with time to first treatment with similar magnitude allowing for equal weighting in the IPS-E score. An independent external consortium successfully confirmed the IPS-E score. Compared with other scores, IPS-E calculation needs the assessment of only 1 molecular variable; namely, the IGHV mutation status, the testing of which is broadly available and standardized, and whose result never changes during the course of disease.


Condoluci A, Terzi di Bergamo L, Langerbeins P, Hoechstetter MA, Herling CD, De Paoli L, Delgado J, Rabe KG, Gentile M, Doubek M, Mauro FR, Chiodin G, Mattsson M, Bahlo J, Cutrona G, Kotaskova J, Deambrogi C, Smedby KE, Spina V, Bruscaggin A, Wu W, Moia R, Bianchi E, Gerber B, Zucca E, Gillessen S, Ghielmini M, Cavalli F, Stussi G, Hess MA, Baumann TS, Neri A, Ferrarini M, Rosenquist R, Forconi F, Foà R, Pospisilova S, Morabito F, Stilgenbauer S, Döhner H, Parikh SA, Wierda WG, Montserrat E, Gaidano G, Hallek M, Rossi D.

Blood. 2020 May 21;135(21):1859-1869. doi: 10.1182/blood.2019003453.

Smolej L, Turcsányi P, Kubová Z, Zuchnická J, Mihályová J, Šimkovič M, Vodárek P, Krčméryová M, Móciková H, Brejcha M, Špaček M; Czech CLL Study Group.

Haematol. 2020 Sep 10. doi: 10.1111/bjh.17074. Epub ahead of print.

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1. Unmutated immunoglobulin heavy variable gene (IGHV)?

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