Evaluation of clinical characteristics, MYD88L265P mutation, CXCR4WHIM mutation and prognosis in Waldenström macroglobulinemia: A single center retrospective study of 93 patients
10.3760/cma.j.issn.0253-2727.2017.06.006
- VernacularTitle: 华氏巨球蛋白血症患者的临床特征、MYD88L265P、CXCR4WHIM突变和预后：单中心93例回顾性分析
- Author:
Xinxin CAO
1
;
Qi MENG
;
Hao CAI
;
Yueying MAO
;
Minghui DUAN
;
Tienan ZHU
;
Wei ZHANG
;
Bing HAN
;
Junling ZHUANG
;
Huacong CAI
;
Miao CHEN
;
Jun FENG
;
Xiao HAN
;
Yan ZHANG
;
Chen YANG
;
Lu ZHANG
;
Daobin ZHOU
;
Jian LI
Author Information
1. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
- Publication Type:Journal Article
- Keywords:
Waldenström macroglobulinemia;
Myeloid Differentiation Factor 88;
Mutation;
Receptors, CXCR4
- From:
Chinese Journal of Hematology
2017;38(6):494-498
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To evaluate the clinical characteristics, MYD88L265P mutation, CXCR4WHIM mutation and prognosis in patients with Waldenström macroglobulinemia (WM).
Methods:The clinical characteristics, International Prognostic Scoring System for symptomatic WM (WPSS) , and overall survival (OS) were retrospectively assayed in 93 patients with newly diagnosed WM at Peking Union Medical College Hospital during January 2000 to August 2016. The MYD88L265P mutation and CXCR4WHIM mutation were tested among 34 patients.
Results:The median age of the 93 patients was 64 years (range, 33-85 years) with a male-to-female ratio of 2.44. According to WPSS, we included 16 (17.2%) low-risk, 44 (47.3%) intermediate-risk and 33 (35.5%) high-risk patients. Eight patients had secondary amyloidosis. With a median follow-up of 44 (1-201) months, the median OS was 84 months. Cox regression multifactor analysis showed WPSS risk group (HR=2.342, 95% CI 1.111-4.950, P=0.025) , whether patients had secondary amyloidosis (HR=5.538, 95% CI 1.958-15.662, P=0.001) and whether patients received new drugs (HR=3.392, 95% CI 1.531-7.513, P=0.003) were independent factors associated with OS. We have investigated the presence of the MYD88L265P and CXCR4WHIM mutation in 34 patients and found that MYD88L265P mutation was occurred in 32 patients (94.1%) and CXCR4WHIM mutation was occurred in 8 patients (23.5%). Seven of 8 patients who harbored CXCR4WHIM-mutated also exhibited the MYD88L265P mutation. Patients with MYD88L265PCXCR4WHIM vs MYD88L265PCXCR4WT presented with more severe anemia, lower platelet level, higher M protein level and more hyper-viscosity syndrome.
Conclusion:WPSS risk group, whether patients had secondary amyloidosis or received new drugs are independent factors for OS in WM. MYD88L265P and CXCR4WHIM mutation, the most common somatic variants in WM, often occur together and impact the clinical presentation.
