症状性华氏巨球蛋白血症患者的临床特征及预后影响因素分析

Clinical features and prognostic factors of patients with symptomatic Waldenstr?m’s macroglobulinemia

目的:探讨症状性华氏巨球蛋白血症(WM)的临床特点及预后因素。方法:收集郑州大学第一附属医院确诊的60例症状性WM患者的临床资料,分析患者临床特征,采用单因素分析和Cox回归模型分析影响患者生存时间的因素。结果:60例症状性WM患者中33(55.0%)例表现为乏力、头晕等贫血相关症状,38(63.3%)例有淋巴结肿大,52(86.7%)例为IgMκ型。55例行骨髓免疫表型检测,其中50(90.9%)例免疫分型为CD19^+、CD20^+、CD10^-、CD5^-、CD79^+、FMC7^+、CD23^-。18例患者中14(77.8%)例MYD88L265P突变阳性。单因素分析结果显示血浆白蛋白水平、乳酸脱氢酶水平、IgA定量、IgM定量、化疗方案均与生存时间有关(P<0.05)。Cox多因素分析显示血浆白蛋白水平是影响症状性WM患者生存时间的独立危险因素,HR(95%CI)为8.632(1.912~38.983)。结论:症状性WM的主要临床表现为贫血相关症状,常见体征为淋巴结肿大,IgMκ型多见。MYD88基因突变率较高。血浆白蛋白水平为影响其预后的独立危险因素。

Aim :To investigate the clinical features and prognostic factors of patients with symptomatic Waldenstrom′s macroglobulinemia(WM). Methods :The clinical data of 60 symptomatic WM patients were collected. The clinical features of the patients were analyzed. Univariate analysis and Cox regression analysis were used to analyze the factors affecting the survival time. Results :Among the 60 patients with symptomatic WM, 33(55.0%) showed anemia-related symptoms such as fatigue and dizziness, 38(63.3%) had lymphadenopathy, and 52(86.7%) had IgMκ style. Out of the 55 patients, 50(90.9%) were immunosuppressed for CD19^+, CD20^+, CD10^-, CD5^-, CD79^+, FMC7^+, and CD23^-. Out of the 18 patients, 14(77.8%) had positive MYD88L265P mutations. Univariate analysis showed that albumin level, lactate dehydrogenase level, IgA quantification, IgM quantification, and chemotherapy regimens were associated with survival time ( P < 0.05). Cox regression analysis showed that albumin level was an independent risk factor for survival time in symptomatic WM patients, with HR (95% CI ) of 8.632(1.912-38.983). Conclusion :The main clinical manifestations of symptomatic WM are anemia-related symptoms.Lymphadenopathy is a common clinical sign, and IgMκ is more common style. The mutation rate of MYD88 gene is high. Plasma albumin level is an independent risk factor that influences the prognosis.