BackgroundThe original description of Castleman’s disease was first reported in 1954 in a series of patients with few or no symptoms but solitary mediastinal lymph node enlargement,which showed the presence of angiofollicular lymph-node hyperplasia with capillary proliferation,hyperplasia of lymphoid follicles,and cellular infiltration of plasma cells,and defined in1956.CD is a rare lymphoproliferative disease,which includes many diseases with similar pathological morphology but different clinical manifestations.According to histopathology,Castleman’s disease can be divided into hyaline vascular type,plasma cell type and mixed type,While unicentric Castleman’s disease(UCD)and multicentric Castleman’s disease(MCD)are distinguished by the feature of distribution of enlarged lymph nodes and organ involvement.Present reports about Castleman’s disease are mainly composed of case reports and clinical characteristics description with small samples,and analysis of large samples about its clinical manifestations,laboratory examination and survival time are rare.Our study retrostpectively collected and analyzed clinical data of 87 patients with CD about clinical manifestations,involving parts and labratory examinations,treatment and prognosis,who are diagnosed in the first affiliated hospital of zhengzhou university through histology.ObjectiveThis study aims to explore the pathological types,clinical manifestations,laboratory indicators,diagnosis and treatment,prognosis and other clinical features of Castleman’s disease,so as to improve clinician’s understanding,diagnosis and treatment level of Castleman’s disease,and provide experience and reference for the standardized diagnosis and treatment of Castleman’s disease.MethodsClinical data of CD patients,confirmed by pathology and immunohistochemistry in our hospital during time from Janurary 1,2012 to July 1,2018,were retrospectively collected according to the inclusion and exclusion criteria.The deadline of follow-up for all patients was December 10,2018.SPSS 22.0 statistical software was used for statistical analysis.Qualitative data were described by rate,while quantitative data were described by mean plus or minus standard deviation,and chi-square test and t-test of two independent samples were used for statistical analysis,α=0.05 was tested.Results1.General information and diagnostic approach55.2% of the whole 86 patients are female,and male patients acount for 44.8%,the age ranges from11 to 73 years old.There is no significant difference between UCD and MCD cases in gender and age(P>0.05).98.4% of UCD is resected to clarify the diagnosis,while 70.8% of MCD is mainly diagnosed by lymph node biopsy,which has significant difference(P<0.001)。2.pathologic type87.3% of UCD cases are hyaline type,both plasma type and mixed type account for 6.3%,while MCD patients are mainly composed of plasma cell type(43.5%),hyaline type accounts for 34.8%,and mixed type is 21.7%(P<0.001)。 Among the immunohistochemical molecules,only the difference of CD20 and CD21 between UCD and MCD has significance(P<0.05),and the rest of all have no sinificance difference between UCD and MCD patients.3.Clinical manifetationsOcurrence rate of fever,fatigue and edema is more frequent in MCD cases than that in UCD cases,which has significant difference(P<0.05),while the occurrence rate of the rest manifestations of them have no signicant difference(P>0.05).4.Localization of Castleman’s diseaseThe rate of involving head and neck,mediastinum,axilla,abdomen,retroperineal area as well as bone and joint system is higher in MCD cases than in UCD cases,which has significant difference(PP<0.05).While the rate of involving thorax and pelvis has no significant difference in both subtypes(P>0.05).5.Labaratory valueThe rate of anemia,abnormal platelets and hypoalbuminemia in MCD cases is higher than that in UCD,which has significant difference(P<0.001).However,the rate of leukocytosis,increased erythrocyte sedimentation rate and C-reactive protein between UCD and MCD cases has no significant difference(P>0.05).6.treatment and prognosisSurgical resection is the main diagnosis and treatment way of UCD(98.4%),while MCD is mainly treated by medicine chemotherapy(88.9%),the difference is significant(P<0.01).The therapeutic drugs applied in patients are various,which contain Glucocorticoid,Cyclophosphamide,Thalidomide,Vincristine,Vindesine,Adriamycin,Pharmorubicin,Etoposide,Rituximab,Melphalan,Vindesine,Cyclosporine,Aclarubicin,Doxorubicin,Pirarubicin,Interferon,Bortezomib,Apatinib,Lenalidomide and so on.Until the follow-up date,we have contacted wih 53 patients in total,the difference of death rate and mean follow-up intervals between UCD and MCD cases has no significant difference(P>0.05).Conclusions1.Castleman disease can occur in all ages,and the gender distribution difference is not significant.Unicentric Castleman’s disease is mainly composed of hyaline vascular type,while the Multicentric Castleman’s disease is mainly composed of plasma cell type,and the difference of immunohistochemical character in both of them is not signicant.Surgical resection is the main diagnosis and treatment way of UCD,while MCD is mainly diagnosed by lymph node biopsy and treated by medicine chemotherapy.2.Compared to UCD,MCD patients have more systemic symptoms and involved more organs easily,whose disease burden is heavier.However,there is no statistically significant difference in follow-up survival and mortality. |