International prognostic score for asymptomatic early-stage chronic lymphocytic leukemia

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Authors

CONDOLUCI A. DI BERGAMO L. T. LANGERBEINS P. HOECHSTETTER M. A. HERLING C. D. DE PAOLI L. DELGADO J. RABE K. G. GENTILE M. DOUBEK Michael MAURO F. R. CHIODIN G. MATTSSON M. BAHLO J. CUTRONA G. KOTAŠKOVÁ Jana DEAMBROGI C. SMEDBY K. E. SPINA V. BRUSCAGGIN A. WU W. MOIA R. BIANCHI E. GERBER B. ZUCCA E. GILLESSEN S. GHIELMINI M. CAVALLI F. STUSSI G. HESS M. A. BAUMANN T. S. NERI A. FERRARINI M. ROSENQUIST R. FORCONI F. FOA R. POSPÍŠILOVÁ Šárka MORABITO F. STILGENBAUER S. DOHNER H. PARIKH S. A. WIERDA W. G. MONTSERRAT E. GAIDANO G. HALLEK M. ROSSI D.

Year of publication 2020
Type Article in Periodical
Magazine / Source Blood
MU Faculty or unit

Faculty of Medicine

Citation
Web https://watermark.silverchair.com/bloodbld2019003453.pdf?token=AQECAHi208BE49Ooan9kkhW_Ercy7Dm3ZL_9Cf3qfKAc485ysgAAA-wwggPoBgkqhkiG9w0BBwagggPZMIID1QIBADCCA84GCSqGSIb3DQEHATAeBglghkgBZQMEAS4wEQQMXRXeaAY25mjozM5SAgEQgIIDnxhMmZAU5cNiFCUDj79Os54hBBpUZ00qooo
Doi http://dx.doi.org/10.1182/blood.2019003453
Keywords chronic lymphocytic leukemia; prognostic score
Description Most patients with chronic lymphocytic leukemia (CLL) are diagnosed with early-stage disease and managed with active surveillance. The individual course of patients with early-stage CLL is heterogeneous, and their probability of needing treatment is hardly anticipated at diagnosis. We aimed at developing an international prognostic score to predict time to first treatment (TTFT) in patients with CLL with early, asymptomatic disease (International Prognostic Score for Early-stage CLL [IPS-E]). Individual patient data from 11 international cohorts of patients with early-stage CLL (n = 4933) were analyzed to build and validate the prognostic score. Three covariates were consistently and independently correlated with TTFT: unmutated immunoglobulin heavy variable gene (IGHV), absolute lymphocyte count higher than 15 x 10(9)/L, and presence of palpable lymph nodes. The IPS-E was the sum of the covariates (1 point each), and separated low-risk (score 0), intermediate-risk (score 1), and high-risk (score 2-3) patients showing a distinct TTFT. The score accuracy was validated in 9 cohorts staged by the Binet system and 1 cohort staged by the Rai system. The C-index was 0.74 in the training series and 0.70 in the aggregate of validation series. By meta-analysis of the training and validation cohorts, the 5-year cumulative risk for treatment start was 8.4%, 28.4%, and 61.2% among low-risk, intermediate-risk, and high-risk patients, respectively. The IPS-E is a simple and robust prognostic model that predicts the likelihood of treatment requirement in patients with early-stage CLL. The IPS-E can be useful in clinical management and in the design of early intervention clinical trials.
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