Clinicopathological features and outcome of chronic lymphocytic leukaemia in Chinese patients

Abstract

// Thomas Sau-Yan Chan 1 , Yuh-Shan Lee 2 , Ilaria Del Giudice 3 , Marilisa Marinelli 3 , Caterina Ilari 3 , Luciana Cafforio 3 , Anna Guarini 4 , Daryl Tan 2 , Colin Phipps 2 , Yeow-Tee Goh 2 , William Hwang 2 , Allan Zhi-Kai Goh 2 , Lisa Lai-Ping Siu 5 , Saliangi Wu 6 , Chun-Yin Ha 7 , Shek-Ying Lin 8 , Chi-Hang Kwok 9 , Chi-Kuen Lau 10 , Kit-Fai Wong 5 , Robin Foà 3 , Yok-Lam Kwong 1 , Eric Tse 1 1 Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong, China 2 Department of Haematology, Singapore General Hospital, Outram, Singapore 3 Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy 4 Department of Molecular Medicine, Sapienza University, Rome, Italy 5 Department of Pathology, Queen Elizabeth Hospital, Hong Kong, China 6 Department of Medicine, Queen Elizabeth Hospital, Hong Kong, China 7 Department of Medicine, Tuen Mun Hospital, Hong Kong, China 8 Department of Medicine, United Christian Hospital, Hong Kong, China 9 Department of Medicine, Princess Margaret Hospital, Hong Kong, China 10 Department of Medicine, Tseung Kwan O Hospital, Hong Kong, China Correspondence to: Eric Tse, email: ewctse@hku.hk Keywords: chronic lymphocytic leukaemia, Chinese, pathological characteristics, clinical outcomes, prognostication Received: November 14, 2016      Accepted: February 13, 2017      Published: March 09, 2017 ABSTRACT Chronic lymphocytic leukaemia (CLL) is uncommon in Chinese population and its biology, genetics and treatment outcome in Chinese patients have not been comprehensively investigated. In this study, we studied the clinicopathological features and outcome of 212 Chinese patients with newly diagnosed CLL in Hong Kong and Singapore. The median age at diagnosis was 64 years. The majority of patients presented with early-stage disease (Binet stage A, 56.1%). Del(13)(q14) was the most frequent abnormality (41.7%) detected by fluorescence in situ hybridization (FISH) analysis. Del(17p) and TP53 gene mutations were detected in 7.8% and 8.2% of patients, respectively. MYD88 mutations were found at a higher frequency (11.5%) than expected. CLL with unmutated variable region of the immunoglobulin heavy chain genes ( IGHV ) occurred in only 31.2% of cases, and was associated with advanced-stage disease (p <0.01) and adverse FISH abnormalities (p<0.01). With a median follow-up of 39 months, the median overall survival (OS) was 108 months. The presence of del(17p) or TP53 mutations was associated with a significantly shorter time to first treatment and an inferior OS (p <0.01). Unmutated IGHV was also associated with a significantly shorter time to treatment (p <0.01). Among patients who required treatment, the median OS and progression-free survival (PFS) were 107 and 23 months, respectively. The presence of del(17p) was associated with a significantly inferior OS and PFS (p <0.01). In summary, Chinese CLL patients had similar genetic aberrations at diagnosis compared with those of Western populations. FISH abnormalities are major factors affecting outcome.