Diagnosis and treatment of Castleman′s disease / 医学研究生学报

ABSTRACT

Objective Castleman′s disease ( CD) is a rare lymphoproliferative disorder, which has a very high misdiagnosis rate according to its lack of clinical and imaging specificity.The study was to improve the recognition, diagnosis and management of this disease by analyzing the clinicopathological characteristics and concluding diagnosis and treatment as well as literature review. Methods Retrospective analysis was conducted on clinical data of 31 CD patients (13 males and 18 females) who were hospitalized in the Affiliated Drum Tower Hosipital to Medical college of Nanjing University from January 2006 to Feburary 2014.Their mean age was (47.4 ±13.3) years.There were 24 cases with unicentric Castleman′s disease (UCD) and 7 cases with multicentric Castleman′s disease ( MCD) .UCD tended to be present in the form of an enlarged and painless mass which generally remained asymptomatic by accidental touch or regular physical examination.MCD was usually associated with constitutional symptoms.All the 24 patients with UCD underwent complete surgical resection.7 patients with MCD received different treatment methods, such as surgical resection, ste-riod and rituximab in combination with chemotherapy ( CHOP) . Results Postoperative pathology confirmed 23 cases were hyaline vascular type while 8 were plasma cell type.25 cases had been followed up (46.3 ±32.3) months after operation, among which 1 UCD case died of pancreatic head carcinoma 13 months after operation, 1 case with MCD plus pemphigus died 2 months after surgical resection of retroperitoneal lymphadenectasis, another MCD case died in 1 month without any treatment.The remaining 22 cases were alive. Conclusion CD is lack of clinical specificity and its diagnosis should be made by pathology.Each subtype differ greatly in prognosis, therefore different treatments should be taken after definitive subtype diagnosis.