Clinico-pathologic Analysis Of 20 Cases Of Castleman

Objective:The study was to improve the recognition of Castleman’s disease by summarizing clinical manifestations,pathological features,treatment and prognosis of 20 patients with Castleman’s disease.Methodology:A retrospective analysis of clinical and pathological data of patients with CD,which diagnosed in Jinling Hospital,Nanjing University School of Medicine during the period from February 2010 to April 2017.Results:All the 20 cases were diagnosed as CD with lymph node biopsy.There were eleven males and nine females whose median age were 41.5(14～73)years old.Clinically,nine cases were UCD and eleven cases were MCD.According to histopathologic typing,twelve were hyaline-vascular type,and eight cases were plasma cell type.Among 9 UCD patients,8 cases were hyaline vascular type,1 cases was plasma cell type.The disease was located in hilar region in 3 cases,in mediastinum in 2 and in retroperitoneal in 4.In patients with UCD,8 cases had no obvious abnormalities in the laboratory and only 1 had autoimmune hemolytic anemia.9 cases of MCD patients with multiple systematic manifestations,including 3 cases with hepatosplenomegaly,4 cases of dropsy of serous cavity,5 cases of renal involvement,1 cases of bone marrow cytology showed eosinophilia of bone marrow,1 cases with allergic granulomatous vasculitis,1 cases with POEMS syndrome.All of MCD had abnormal laboratory examination results,including 7 cases had anemia,8 cases had elevated CRP,8 cases had hypoalbumemia,5 cases had increased erythrocyte sedimentation rate,2 cases had increased serum IL-6 level,1 cases of immunofixation electrophoresis of monoclonal IgA and free lambda lambda chain.4 cases of antinuclear antibody and antineutrophil cytoplasmic antibody(ANCA)in patients were positive,2 cases showed anti nuclear antibody(ANA)positive,1 cases were positive for anti SSA antibody,1 cases were both positive for ANA and ANCA.All 5 patients with renal impairment were in the MCD group,including 4 males and 1 females,with a median age of 42 years,all of them showed positive proteinuria and impaired renal function.The pathological types were 3 cases of plasma cell type and 2 cases of hyalinevascular type.Renal biopsy was performed in 2 patients.The pathological changes were glomerular membrane proliferative lesions and renal amyloidosis respectively.All patients underwent CT scans which showed homogeneous soft tissue mass,and calcification was observed in 3 cases.Three patients underwent 18F-FDG PET/CT examination,and demonstrated slightly FDG uptake in multiple lymph nodes.All of the 8 patients with UCD underwent complete resection and only one took comprehensive treatment with radiotherapy and chemotherapy.All patients survived with the exception of 1 patient who was lost.In the 11 patients with MCD,9 cases received prednisone,CHOP,CTD or VRD chemotherapy,2 cases of complete remission,partial remission in 2 cases,4 cases of disease progression,death in 1 cases,The remaining 2 patients with retroperitoneal lesions received glucocorticoid single drug and CHOP chemotherapy after surgery,2 cases of patients with disease progression.Conclusion:The diagnosis of Castleman’s disease mainly depends on pathological examination.UCD patients often have no obvious clinical symptoms,and the laboratory tests are normal.Complete surgical resection is the standard treatment for unicenrtic Castleman disease and had a high cure rate with surgical excision.MCD oftenmanifested with constitutional symptoms,and sometimes associated with renal impairment which renal biopsy reveals various lesions.Comprehensive treatment are usually used in multicentric Castleman disease.