Journal
HAEMATOLOGICA
Volume 107, Issue 4, Pages 868-876Publisher
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.2021.278304
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Funding
- Associazione Italiana per la Ricerca sul Cancro (A.I.R.C.) [IG-25024]
- Gilead fellowship program 2018
- AIRC [21198]
- University of Ferrara
- Ministero dell'Istruzione, dell'Universita e della Ricerca PRIN 2015 [2015ZMRFEA]
- University of Padua - ONLUS Ricerca per Credere nella Vita (RCV) odv, Padua, Italy
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Complex karyotype (CK) at chronic lymphocytic leukemia (CLL) diagnosis is a negative biomarker of adverse outcome. The impact of CK on the risk of developing Richter syndrome (RS) is unknown. A multicenter real-life retrospective study was conducted to test its prognostic impact. The results demonstrate that CK landscape at CLL diagnosis allows the risk of RS transformation to be refined and a prognostic model is established.
Complex karyotype (CK) at chronic lymphocytic leukemia (CLL) diagnosis is a negative biomarker of adverse outcome. Since the impact of CK and its subtypes, namely type-2 CK (CK with major structural abnormalities) or high-CK (CK with >= 5 chromosome abnormalities), on the risk of developing Richter syndrome (RS) is unknown, we carried out a multicenter real-life retrospective study to test its prognostic impact. Among 540 CLL patients, 107 harbored a CK at CLL diagnosis, 78 were classified as CK2 and 52 as high-CK. Twenty-eight patients developed RS during a median follow-up of 6.7 years. At the time of CLL diagnosis, CK2 and high-CK were more common and predicted the highest risk of RS transformation, together with advanced Binet stage, unmutated (U)-IGHV, 11q-, and TP53 abnormalities. We integrated these variables into a hierarchical model: high-CK and/or CK2 patients showed a 10-year time to RS (TTRS) of 31%; U-IGHV/11q-/TP53 abnormalities/Binet stage B-C patients had a 10-year TTRS of 12%; mutated (M)-IGHV without CK and TP53 disruption a 10-year TTRS of 3% (P<0.0001). We herein demonstrate that CK landscape at CLL diagnosis allows the risk of RS transformation to be refined and we recapitulated clinico-biological variables into a prognostic model.
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