Clinicopathological features and outcome of chronic lymphocytic leukaemia in Chinese patients
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Thomas Sau-Yan Chan1, Yuh-Shan Lee2, Ilaria Del Giudice3, Marilisa Marinelli3, Caterina Ilari3, Luciana Cafforio3, Anna Guarini4, Daryl Tan2, Colin Phipps2, Yeow-Tee Goh2, William Hwang2, Allan Zhi-Kai Goh2, Lisa Lai-Ping Siu5, Saliangi Wu6, Chun-Yin Ha7, Shek-Ying Lin8, Chi-Hang Kwok9, Chi-Kuen Lau10, Kit-Fai Wong5, Robin Foà3, Yok-Lam Kwong1, Eric Tse1
1Department of Medicine, The University of Hong Kong, Queen Mary Hospital, Hong Kong, China
2Department of Haematology, Singapore General Hospital, Outram, Singapore
3Department of Cellular Biotechnologies and Hematology, Sapienza University, Rome, Italy
4Department of Molecular Medicine, Sapienza University, Rome, Italy
5Department of Pathology, Queen Elizabeth Hospital, Hong Kong, China
6Department of Medicine, Queen Elizabeth Hospital, Hong Kong, China
7Department of Medicine, Tuen Mun Hospital, Hong Kong, China
8Department of Medicine, United Christian Hospital, Hong Kong, China
9Department of Medicine, Princess Margaret Hospital, Hong Kong, China
10Department of Medicine, Tseung Kwan O Hospital, Hong Kong, China
Eric Tse, email: [email protected]
Keywords: chronic lymphocytic leukaemia, Chinese, pathological characteristics, clinical outcomes, prognostication
Received: November 14, 2016 Accepted: February 13, 2017 Published: March 09, 2017
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.
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