多中心Castleman病的临床诊断学特征并文献复习

Diagnosis and literature review of multicentric castleman disease

目的探讨多中心型Castleman病(MCD)的临床诊断学特征。方法回顾性分析2016年5月29日济宁医学院附属医院呼吸内科收治的1例混合型MCD患者的临床特点,并复习相关文献,总结MCD患者的临床诊断学特点。结果患者因发热、乏力10余天入院,行正电子发射计算机断层显像(PET/CT)提示双侧颈部、锁骨上区、纵隔内、双侧腋下、腹膜后、腹腔、盆腔、双侧腹股沟多发淋巴结肿大,代谢不同程度增高,双侧胸腔积液并双肺下叶膨胀不全,盆腔积液,全身骨弥漫性代谢增高,符合Castleman病的PET/CT表现。行颈部淋巴结活检,病理学示混合型CD。免疫组化:CD3T淋巴细胞(+),CD20B淋巴细胞(+),CD21滤泡FDC呈网状(+),Ki-67滤泡阳性细胞数比例高于75%,滤泡间区阳性细胞数5%~10%,CD34血管(+)。结论本病影像学表现有一定特征,早期行活检组织病理学检查有助于诊断。

Objective To explore the diagnostic features of muhicentric castleman disease（MCD）. Methods The clinical features of a MCD patient, who was diagnosed in respiratory department of the Affiliated Hospital of Jining Medical University in 2016 were analyzed.Relevant literatures were reviewed and characteristics of clinical diagnosis of MCD were summarized.Results A 29 year old woman was hospitalized because of fever, fatigue over ten days. Positron emission tomography computed tomography （PET/CT） showed bilateral neck, clavicle, mediastinal, bilateral axillary, retroperitoneal, abdominal, pelvic, bilateral inguinal nodes enlargement and metabolism were increased in different degrees.Bilateral pleural effusion and atelectasis,pelvic effusion bone metabolism were increased, the features were in accordance with the PET/CT manifestation of CD. Mix-MCD was diagnosed by cervical lymph node biopsy. Immunohistochemical showed CD3T lymphocytes （ ＋ ）, CD20B lymphocytes （ ＋ ）, CD21 follicular FDC showing reticular （ ＋ ）, the percentage of Ki-67 positive cells was higher than 75%, the percentage of positive cells in the follicular area was 5% - 10%, CD34 blood vessels （＋） .Conclusion The imaging manifestations of the disease manifests certain characteristics, early biopsyhistopathological examination is helpful for diagnosis.