Castleman病合并贫血的临床分析

Clinical Analysis of Anemia in Castleman Disease

目的:本研究旨在探讨Castleman病(CD)合并贫血的特征。方法:回顾性研究33例CD患者的临床资料,分析贫血发生率和临床特征,探讨贫血类型及其治疗和疗效。结果:13/33(39%)例CD初诊时有贫血表现,多为轻度,正细胞正色素型。多中心CD(multicentric CD,MCD)贫血发生率高于单中心CD(unicentric CD,UCD)(85%vs 10%)(P<0.001)。与无贫血者相比,CD合并贫血者多具有系统性表现,且血沉、炎症指标(C反应蛋白和纤维蛋白原)水平、多克隆免疫球蛋白增多及自身抗体阳性的发生率均升高(P均<0.05)。进一步分析贫血类型发现,除2例自身免疫性溶血性贫血(AIHA)和1例脾亢继发贫血外,余10例均具有慢性病贫血(ACD)的特征,其血红蛋白水平与C反应蛋白(CRP)及纤维蛋白原均呈负相关(r分别为-0.917、-0.717,P均<0.001)。UCD合并贫血者经肿瘤切除后治愈,MCD合并贫血者经免疫和/或化疗治疗后贫血好转。结论:贫血为MCD常见的系统性表现,亦可见于UCD。除少数表现为AIHA外,多数具有ACD特征。CD合并贫血的治疗以治疗原发病为主。

Objective： This study was to explore the characteristics of anemia in Castleman disease （CD）. Methods： Clinical data were collected retrospectively to analyze the prevalence and characteristics of CD with anemia were analysed retrospectively, and different types of anemia and their therapeutic effects were evaluated. Results： Anemia was observed in 13/33 （39%） newly diagnosed CD patients, most of them was mild and normocytic. Incidence of anemia in multicentric CD （MCD） was higher than that in unicentric CD （UCD） （ 85 % vs 10% , P 〈 0.001 ）. Most of CD patients with anemia presented systematic manifestations; moreover, they had higher levels of erythrocyte sedimentation and inflammatory indices, higher incidence of polyclonal hyperimmunoglobulinemia, and higher positive rate of autoantibodies than those without anemia （ P 〈 0.05 ）. Except for 2 cases of autoimmune hemolytic anemia （AIHA） and 1 case of anemia secondary to hypersplenism, the anemia in the other 10 patients exhibited features similar to anemia of chronic disease （ACD）, whose hemoglobin levels were negatively correlated with the serum levels of C reactive protein and fibrinogen （r -0. 917 and -0. 717, respectively,P 〈0.001 ）. Anemia in UCD was cured by the removal of tumor. Yet, anemia in MCD was improved after systemic treatment with immunotherapy and/or chemotherapy. Conclusions： anemia with an inflammatory and immunologic mechanism presents as a common symptom in MCD, but also can be observed in UCD. In addition to occasional AIHA, anemia associated with CD mainly presents characteristics of ACD. Treatment for anemia in CD is mainly based on the control of primary disease.