The Clinical Characteristics,Pathological Features,Therapies And Prognostic Factors Of Castleman Disease

Objective:Due to the limitation of Castleman disease(CD),especially HIV-/HHV8-multicentric CD(iMCD),lack the world-wild multi-ethnic research in clinical features and treatment options.It is urgent to optimize the diagnosis and treatment to improve the prognosis.Methods:We retrospectively collected the clinical information of 428 patients with HIV and HHV-8 negative CD from China and the United State with a 15-year follow-up.We focus on the clinical features,pathological diagnosis,classification,treatment plan,and risk factors.In addition,we also reviewed 44 cases of CD in our hospital since 2007.Results:Analysis of 428 cases:(1)iMCD accounted for 42.06% and showed more systemic inflammation and organ function damage compared with Unicentric CD(UCD).It should be noted that one patient with family history.Compared to the Plasma variant(PC)with the Hyaline vascular variant(HV),it is a certain proportion of crossover.HV type accounted for 76.58% in UCD,and PC type accounted for 71.69%in iMCD.(2)iMCD was used to treat with hormone,combined chemotherapy,Rituximab,Siltuximab and observation waiting,with different efficacy but no significant difference was observed.The total response rate and 5-year overall survival rate of 93.5% of UCD patients were significantly better than that of iMCD,while the failure rate was significantly lower.(3)A multivariate analysis revealed that PC subtype,hepatomegaly and/or splenomegaly,hemoglobin ≤ 80 g/L,and albumin ≤ 30 g/L were independent prognostic factors of CD for OS.The model of iMCD revealed that age >60 years,hepatomegaly and/or splenomegaly,and hemoglobin ≤ 80 g/L were independent risk factors.Single-center case analysis in China:(1)the clinical phenotype and inflammatory indicators of UCD and MCD in our center were heterogeneous,it was similar to our study.(2)Currently,chemotherapy is still the main treatment with good efficacy for iMCD(6/13).Conclusion:CD has strong heterogeneity:(1)Systemic inflammation and organ function injury in UCD patients were significantly lighter than those in iMCD,and surgical resection was the standard treatment with good efficacy and prognosis.(2)iMCD presents heterogeneous clinical manifestations,ranging from asymptomatic to systemic inflammatory response and organ function injury,with varying degrees of severity.Siltuximab is the main treatment in foreign,while hormone,combined chemotherapy,and rituximab are the main treatment in China,and there is no priority choice.(3)Pathological type,anemia,decreased albumin,liver and/or splenomegaly and age may affect the survival of patients.We should pay attention to correct anemia,hypoproteinemia,and liver-protecting enzymes,which will help to improve the curative effect and prognosis.