以腹水为主要特征的Castleman病16例  

Castleman disease characterized by ascites:report of sixteen cases

作　　者：朱旦华 卢放根[2] Zhu Danhua;Lu Fanggen(Department of Gastroenterology,Hunan Provincial People's Hospital,The First Affiliated Hospital of Hunan Normal University,Changsha 410000,Hunan Province,China;Department of Gastroenterology,The Second Xiangya Hospital of Central South University,Changsha 410000,Hunan Province,China)

机构地区：[1]湖南师范大学附属第一医院消化内科,湖南长沙410000 [2]中南大学湘雅二医院消化内科,湖南长沙410000

出　　处：《中华临床医师杂志(电子版)》2024年第5期462-473,共12页Chinese Journal of Clinicians(Electronic Edition)

摘　　要：目的总结以腹水为主要特征Castleman病的临床特点及诊治过程,旨在提高对此类Castleman病的诊治认识。方法回顾性分析2006年1月~2023年1月湘雅二医院及湖南省人民医院收治的6例以腹水为主要特征Castleman病的临床资料,并对至今国内外报道的10例以腹水为起病特点Castleman病进行文献复习。结果本文16例患者,男性10例,女性6例。16例均以腹胀为首发临床表现,伴有多发淋巴结肿大及脾脏增大。6例本院病例血红蛋白为(95.8±15.1)g/L,白蛋白为(31.7±5.0)g/L,血沉为(28.8±9.83)mm/h。腹水细胞总数为(370±190.5)×10^(6)/L,以单核细胞为主,腹水白蛋白定量为(25.4±5.6)g/L。10例文献报道患者血红蛋白为(103.8±15.5)g/L,白蛋白为(30.3±4.8)g/L,血沉为(34.1±8.6)mm/h。腹水细胞总数为(184.4±110.1)×10^(6)/L,以单核细胞为主,腹水白蛋白定量为(22.9±7.5)g/L。16例腹水CEA及细胞学检查阴性。16例病理分型为混合型5例,透明血管型4例,浆细胞型5例,未分型2例。4例单用激素治疗,症状可缓解。6例行CHOP方案(环磷酰胺+阿霉素+长春新碱+泼尼松)治疗,随访1年,腹水多数可控制。结论以腹水为主要临床特征的Castleman病有以下几个特征:男性病例多见;首发表现为腹胀,可伴有轻度贫血、低蛋白血症和血沉增快;腹水以低细胞数、较高蛋白定量和李凡他试验阳性为其特点。Castleman病诊断依据为病理组织学检查。激素和CHOP方案短期缓解症状较好,但长期效果差。Objective To investigate the clinical features,diagnosis,and treatment of Castleman disease characterized by ascites.Methods The data of six patients with Castleman disease characterized by ascites admitted to our hospital from January 2006 to January 2023 and ten patients identified from the literature were reviewed.Results Of the total of 16 cases,10 were male and 6 were female.All the 16 cases had abdominal distension as the first manifestation.All the 16 cases showed many enlarged lymph nodes and spleen enlargement.For the six cases in our hospital,the average hemoglobin was(95.8±15.1)g/L,albumin was(31.7±5.0)g/L,erythrocyte sedimentation rate was(28.8±9.83)mm/h,the number of cells in ascites was(370±190.5)×10^(6)/L,the predominant cell type in ascites was monocytes,and the level of albumin in ascites was(25.4±5.6)g/L.For the 10 patients identified from the literature,the hemoglobin was(103.8±15.5)g/L,albumin in 9 cases was(30.3±4.8)g/L,ESR was(34.1±8.6)mm/h,the number of cells in ascites was(184.4±110.1)×10^(6)/L,the predominant cell type in ascites was monocytes,and the level ofalbumin in ascites was(22.9±7.5)g/L.Carcinoembryonic antigen in ascites and ascites cytology were negative in all the 16 cases.The pathological classification was mixed type in 5 cases,transparent vascular type in 4,plasma cell type in 5,and unknown type in 2.Corticosteroids were used for treatment in 4 cases,and the symptoms could be alleviated.Six patients underwent chemotherapy with the CHOP(cyclophosphamide,adriamycin,vincristine and prednisone)regimen;during the 1-year follow-up period,the ascites of patients could be controlled.The long-term prognosis of patients with Castleman disease was poor.Conclusion Castleman disease patients with ascites as a predominant clinical feature are characterized by:a high male/female ratio;an onset symptom of abdominal distension with possible manifestations like mild anemia,hypoalbuminemia,and elevated ESR;and low cell count and relatively high protein in ascites and positive Rival

关 键 词：CASTLEMAN病 腹水 诊断 治疗 

分 类 号：R551.2[医药卫生—血液循环系统疾病]