External validation of International Prognostic Score for asymptomatic early stage chronic lymphocytic leukaemia and proposal of an alternative score
Language English Country England, Great Britain Media print-electronic
Document type Journal Article, Research Support, Non-U.S. Gov't
Grant support
PROGRES Q40/08
Univerzita Karlova v Praze
FNHK, RVO 0017906
Ministerstvo zdravotnictví České republiky
VFN, RVO 64165
Ministerstvo zdravotnictví České republiky
Ministry of Health
PubMed
33280081
DOI
10.1111/bjh.17074
Knihovny.cz E-resources
- Keywords
- FISH, IGHV, chronic lymphocytic leukaemia, prognosis, time to first-line therapy,
- MeSH
- Time-to-Treatment statistics & numerical data MeSH
- Chromosome Aberrations statistics & numerical data MeSH
- Leukemia, Lymphocytic, Chronic, B-Cell blood diagnosis mortality pathology MeSH
- Genes, Immunoglobulin Heavy Chain genetics MeSH
- In Situ Hybridization, Fluorescence methods MeSH
- Cohort Studies MeSH
- Humans MeSH
- Lymphadenopathy diagnosis MeSH
- Palpation methods MeSH
- Lymphocyte Count methods MeSH
- Prognosis MeSH
- Disease Progression MeSH
- Risk Factors MeSH
- Aged MeSH
- Neoplasm Staging methods MeSH
- Research Design trends MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Czech Republic epidemiology MeSH
Most patients with chronic lymphocytic leukaemia (CLL) are nowadays diagnosed without any symptoms and do not require therapy. A prognostic score identifying patients within this large group who are at high risk of disease progression would be highly beneficial. The recently published International Prognostic Score for Early asymptomatic patients (IPS-E) uses combination of absolute lymphocyte count (ALC) >15 × 109 /l, palpable lymphadenopathy, and unmutated immunoglobulin heavy-chain variable-region (IGHV) gene to predict the time to first-line therapy (TTFT). Patients at low, intermediate, and high risk had estimated 5-year TTFT of 8%, 28%, and 61%. We performed an external validation of the IPS-E score using an unselected, consecutive group of 130 Binet A patients. The 5-year TTFT was 11%, 36%, and 78% (C-statistic 0·74). Furthermore, we propose an alternative system (AIPS-E) using cytogenetic aberrations instead of palpable lymphadenopathy. This system yielded 5-year TTFT of 14%, 40%, and 72%. These results were externally validated in 388 Binet A patients from five Czech centres; the 5-year TTFT was 16%, 37%, and 80% (C-statistic 0·74). In conclusion, we have successfully validated the IPS-E score for patients with early stage CLL. In addition, we propose a modified scoring system, the AIPS-E, combining IGHV, fluorescence in situ hybridisation, and ALC.
1st Department of Medicine Haematology University General Hospital Prague Czech Republic
Department of Haematology Oncology Center Nový Jičín Czech Republic Prague Czech Republic
Department of Haematology Oncology University Hospital Olomouc Czech Republic
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Binet JL, Auquier A, Dighiero G, Chastang C, Piguet H, Goasguen J, et al. A new prognostic classification of chronic lymphocytic leukemia derived from a multivariate survival analysis. Cancer. 1981;48:198-206.
Rai KR, Sawitsky A, Cronkite EP, Chanana AD, Levy RN, Pasternack BS. Clinical staging of chronic lymphocytic leukemia. Blood. 1975;46:219-34.
Condoluci A, Terzi di Bergamo L, Langerbeins P, Hoechstetter MA, Herling CD, De Paoli L, et al. International prognostic score for asymptomatic early-stage chronic lymphocytic leukemia. Blood. 2020;135:1859-69.
Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Dohner H, et al. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the International Workshop on Chronic Lymphocytic Leukemia updating the National Cancer Institute-Working Group 1996 guidelines. Blood. 2008;111:5446-56.
Rosenquist R, Ghia P, Hadzidimitriou A, Sutton LA, Agathangelidis A, Baliakas P, et al. Immunoglobulin gene sequence analysis in chronic lymphocytic leukemia: updated ERIC recommendations. Leukemia. 2017;31:1477-81.
Rack KA, van den Berg E, Haferlach C, Beverloo HB, Costa D, Espinet B, et al. European recommendations and quality assurance for cytogenomic analysis of haematological neoplasms. Leukemia. 2019;33:1851-67.
van den Brekel MW, Castelijns JA. What the clinician wants to know: surgical perspective and ultrasound for lymph node imaging of the neck. Cancer Imaging. 2005;5(Special Issue A):S41-9.
Baatenburg de Jong RJ, Rongen RJ, Lameris JS, Harthoorn M, Verwoerd CD, Knegt P. Metastatic neck disease. Palpation vs ultrasound examination. Arch Otolaryngol Head Neck Surg. 1989;115:689-90.
Muntanola A, Bosch F, Arguis P, Arellano-Rodrigo E, Ayuso C, Gine E, et al. Abdominal computed tomography predicts progression in patients with Rai stage 0 chronic lymphocytic leukemia. J Clin Oncol. 2007;25:1576-80.
Gentile M, Cutrona G, Molica S, Ilariucci F, Mauro FR, Di Renzo N, et al. Prospective validation of predictive value of abdominal computed tomography scan on time to first treatment in Rai 0 chronic lymphocytic leukemia patients: results of the multicenter O-CLL1-GISL study. Eur J Haematol. 2016;96:36-45.
Dohner H, Stilgenbauer S, James MR, Benner A, Weilguni T, Bentz M, et al. 11q deletions identify a new subset of B-cell chronic lymphocytic leukemia characterized by extensive nodal involvement and inferior prognosis. Blood. 1997;89:2516-22.
Zenz T, Eichhorst B, Busch R, Denzel T, Habe S, Winkler D, et al. TP53 mutation and survival in chronic lymphocytic leukemia. J Clin Oncol. 2010;28:4473-9.
Hallek M, Cheson BD, Catovsky D, Caligaris-Cappio F, Dighiero G, Dohner H, et al. Guidelines for diagnosis, indications for treatment, response assessment and supportive management of chronic lymphocytic leukemia. Blood. 2018;131:2745-60.