Clinical Manifestation,Trerapies And Prognostic Factors Of Idiopathic Multicentric Castleman Disease

Background:Idiopathic multicentric castleman disease(iMCD)is a rare inflammatory lymph node proliferative disease.The etiology and pathogenesis of Castleman’s disease are not very clear.The diagnosis of iMCD mainly depends on the histopathology of biopsy tissues and the exclusion of malignant tumors,rheumatic immune system diseases and infectious diseases.Due to the great heterogeneity of CD in clinical manifestations,pathological features,laboratory characteristics and therapeutic effects,and due to the rarity of the disease,there is currently a lack of large sample prospective data at home and abroad to support treatment selection and prognosis model.Objects:The clinical characteristics,laboratory characteristics,treatment and efficacy,survival time and outcome of iMCD were evaluated.The risk prognostic factors affecting the survival time and outcome of iMCD patients were searched,and risk stratification model of iMCD patients was made accordingly,which was used to predict the initial response of iMCD patients to treatment,the possibility of recurrence and the overall survival rate during follow-up.Methods:In this study,a total of 176 iMCD patients from China and the United States admitted since 1 January 2005 and 28 February 2017.The clinical manifestations,responses to treatment regimens,survival outcomes and prognosis of the disease were analyzed retrospectively.Results:(1)iMCD has great heterogeneity in clinical symptoms,histopathology and laboratory results.(2)Treatment was based on the severity of clinical symptoms,varying from watchful waiting to curative-intent combination chemotherapy regimens(CHOP chemotherapy regimens or CHOP plus rituximab(R-CHOP)).The study found that the rituximab remission rate is significantly higher than use prednisone,but recurrence often happen.In patients with refractory relapse,siltuximab or tocilizumab and other targeted drugs showed a strong ability to improve clinical symptoms and effectively extended the overall survival of iMCD.(3)In iMCD patients,univariate analysis suggested that age>40 years,plasma cell subtypes,hemoglobin<80g/L,hepatomegaly and/or splenomegaly,and pleural effusion were risk prognostic factors for Overall survival,predicting worse survival outcomes,including initial response to treatment,likelihood of recurrence,and overall survival at follow-up.(4)The five risk prognostic factors were used as the scoring basis,each risk factor score was 1 point,which was divided into low-risk(0-1),intermediate-risk(2-3)and high-risk groups(4-5).The iMCD-IPI risk stratification model predicted survival outcomes in iMCD patients,including initial response to treatment,likelihood of recurrence,and overall survival at follow-up.Conclusion:iMCD has greater heterogeneity and tends to have more severe inflammatory responses and laboratory results.iMCD treatment varied widely,with asymptomatic patients more often choosing "watchful waiting",and those with more symptoms using anti-IL-6 monoclonal antibody or combined chemotherapy more often.Univariate analysis suggested that age >40 years,plasma cell subtype,hemoglobin <80g/L,hepatomegaly and/or splenomegaly,and pleural effusion were risk prognostic factors affecting overall survival of iMCD.According to the above five risk prognostic factors as the basis of scoring,it was divided into low-risk,intermediate-risk and high-risk groups.The iMCD-IPI score accurately predicted our findings,including initial response to treatment,likelihood of recurrence,and overall survival at follow-up.Our study will help iMCD patients in clinical work to choose a more appropriate treatment plan and reasonable treatment path,improve the survival and prognosis of patients,and deepen our understanding of iMCD,which is a rare malignant tumor.