OBJECTIVES:Castleman’s disease(CD),a rare angiofollicular lymph node hyperplasia,was easily confused with other diseases,due to complex and diverse clinical manifestations.And there was little data regarding CD in Chinese people.Therefore the study was conducted to explore the characteristics of CD and provide evidence for its diagnosis and treatment.METHODS:30 CD patients aged 7-71 years were selected in the study which was histologically proved CD at Qilu Hospital of Shandong University from January 2013 to December 2017.The average age was(43.5±18.1)years.There were 13 men with an average age of(44.9±14.9)years and 17 women with an average age of(42.4±20.6)years.According to CD diagnostic criteria of 2015 NCCN Guide,all patients were divided into two groups:unicentric Castleman’s disease(UCD)and multicentric Castles man’s disease(MCD).Gender,age,history of smoking and drinking,chief complant,clinical presentation,lymph node distribution,imaging examination,laboratory examination,histological feature,treatment and prognosis of all patients were analyzed by SPSS 21.0 software.Fisher exact test was choosed for intergroup comparison.RESULTS:(1)General information:In 13 men,there were 0 patient(0%)at the age of ≤ 17,5 patients(38.5%)of 18-40,6 patients(46.2%)of 41-65,and 2 patients(15.4%)of ≥ 66.In 17 women,there were 2 patient(11.8%)at the age of ≤ 17,5 patients(29.4%)of 18-40,7 patients(41.2%)of 41-65,and 3 patients(17.6%)of≥ 66.There was no statistical significance between sex and age(P>0.05).(2)Clinical characteristics:There were 26 patients with UCD and 4 with MCD.There were diverse symptoms,including fever(8.3%),edema(8.3%),anemia(33.3%),leukopenia(33.3%),hypoalbuminia(25.0%),abdominal distention and pain(41.7%),anorexia(16.6%),numbness of extremities(8.3%),hoarseness(8.3%).UCD consisted of 18 asymptomatic patients,8 symptomatic patients.MCD consisted of 4 symptomatic patients.There was statistical significance between clinical classification and symptoms(P<0.05).(3)Location:UCD was localized to one site,including neck(25.9%),mediastinum(17.9%),retroperitoneum(17.9%),abdominal cavity(15.4%),groin(7.7%),armpit(5.2%)pelvic cavity(2.6%),orbit(2.6%)and throat(2.6%).MCD was localized to two or more sites.(4)Histopathological characteristics:In histological feature,hyaline-vascular type was identified in 22 patients(84.6%)with UCD and in 1 patient with MCD(25%).Conversely,plasma cell type was observed in 3 patients(11.5%)with UCD and in 3 patients with MCD(75%).Mixed type was identified in 1 patient with UCD(3.8%).There was statistical significance between clinical classification and symptoms(P<0.05).(5)Treatment:All patients underwent operation for resection of the tissue mass.26 UCD patients choosed complete resection.4 MCD choosed lymph node biopsy.After operation,2 MCD patients receiving combined chemotherapy,which consist of CHOP regimen.CONCLUSION:CD is a chronic progressive disease,which has nothing to do with gender and age.UCD is more common than MCD.CD has no specific clinical symptoms and is easy to be misdiagnosed.The symptoms of UCD is related to the location of the mass,while MCD is complex,diverse and severe.CD can occur in any part of the body,regardless of any age,sex and pathological type.The diagnosis of CD requires histopathological examination.Surgery is an effective method for UCD treatment and can survive for a long time after operation.MCD can be combined with chemotherapy with poor prognosis. |