The clinical features of patients with lymphoplasmacytic diseases harboring MyD88 L265P mutation
10.3760/cma.j.issn.0253-2727.2016.12.009
- VernacularTitle:伴MyD88 L265P突变淋巴浆细胞疾病患者的临床特征分析
- Author:
Yuan REN
1
,
2
,
3
,
4
;
Biqi ZHOU
;
Yang XU
;
Chengcheng FU
;
Hongjie SHEN
;
Zixuan DING
;
Depei WU
Author Information
1. 215006 苏州大学附属第一医院、江苏省血液研究所
2. 卫生部血栓与止血重点实验室
3. 血液学协同创新中心
4. 苏州大学造血干细胞移植研究所
- Keywords:
Myeloid differentiation factor 88;
DNA mutation;
Lymphoproliferative disorders;
Clinical characteristics
- From:
Chinese Journal of Hematology
2016;37(12):1054-1059
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore the clinical features oflymphoplasmacytic diseases with MyD88 L265P mutation.Methods To analyze the distribution of MYD88 L265P mutation in patients with lymphoplasmacytic diseases by using of ARMS PCR-CE.Results There were 25(30.9％) MyD88 L265P mutated patients in 81 patients.The mutation was frequently observed in 14 patients with WM (77.8％,14/ 18),2 patients with lymphoplasmacytic lymphoma (66.7％,2/3),1 acute lymphocytic leukemia patient (50.0％,1/2),3 multiple myeloma patients (30.0％,3/10),1 patient with monoclonal gammopathy of undetermined significance (25％,1/4),3 patients with chronic lymphocytic leukemia (13.0％,3/23) and 1 lymphoma patient (4.8％,1/21).20 (80％,20/25) patients were identified with IgM subtype.Compared with wild-type group of 56 cases,mutated patients were older (median age:67 years vs 55 years,P＜ 0.001),with lower WBC count (median count:5.23 × 109/L vs 10.80× 109/L,P=0.001),lower HGB level (median count:85 g/L vs 119 g/L,P＜0.001).Conclusion MyD88 L265P mutation was mainly observed in patients with IgM subtype lymphoplasmacytic diseases,and Waldenstrom's macroglobulinemia was the most common disease.Compared with the wild-type group,patients with MyD88 L265P mutation were older and had lower WBC count,lower level of HGB.However,further studies were needed to test the prognostic value of MyD88 L265P mutation.
