Clinical,Pathological Features And Treatment Analysis Of 5 Cases Of Multicenter Castleman's Disease And Literature Review

Objective : Castleman's disease(CD)is a rare lymphoproliferative disorder.Because of its low incidence,the incidence is still unclear.Its clinical manifestations are not specific,and there is no uniform standard for treatment.This article summarizes the clinical data of 5 patients with multicenter Castleman's disease(MCD),summarizes the clinical features,diagnosis and prognosis of CD to improve the understanding of CD.Methods : To analyze and summarize the Characteristics of clinical data of 5 patients with multicenter Castleman's disease(MCD)admitted to the Affiliated Hospital of Tongji Medical College from Huazhong University of Science and Technology from December 2008 to November 2018.Using fisher test to analyze clinical data of patients and analyze clinical characteristics,and review relevant literature.Results: 1)Castleman disease is rare.In the case of 5 cases of multicenter Castleman,3 cases were male and 2 were female.The male to female ratio was 1.5:1,and the median age was 49(24-64)years old.2)5 cases of cervical lymph nodes,4 cases of axillary and inguinal lymph nodes,1 case of subclavian lymph nodes,4 cases of abdominal lymph nodes,and 1 case of mediastinal lymph nodes.3)There were 3 cases of fever and hepatosplenomegaly in this group,and 1 case of ascites.There were 1case of chronic hepatitis B with 1 case of thyroid nodules and 1 case of rheumatoid arthritis.Five cases of HIV were negative.4)Five patients with MCD were diagnosed with anemia in 4 cases,thrombocytopenia in 2 cases,increased in 1 case,5 cases with hsCRP abnormalities,3 cases with increased erythrocyte sedimentation rate(ESR),2cases with hypoproteinemia,and 5 cases with decreased A/G.2 cases of ?2microglobulin abnormalities,1 case of rheumatoid factor(RF)abnormalities.In one case of immunofixation electrophoresis,anti-IgG,IgA,IgM and anti-kappa,?-formed fixation bands were increased in polyclonal.In one case of immunofixation electrophoresis,the anti-IgG,IgA and anti-kappa,?-formed fixation bands wereincreased in multiples.5)CD imaging has no specific performance and can be used to determine the location and size of the lesion,as well as to evaluate the patient's response to chemotherapy.CT showed multiple lymphadenopathy,and the lesion was a clearly defined "elliptical" soft tissue mass with uniform density.PET-CT can observe enlarged lymph nodes,abnormal concentration of radioactive distribution in the lesions,and abnormal increase in lymph node metabolism.6)CD diagnosis relies on pathological biopsy.All patients in this group were diagnosed by lymph node biopsy.3 cases were plasma cell type and 2 cases were transparent blood vessel type.7)At present,there is no uniform standard for the treatment of CD.In this group of cases,one case of multi-center Castleman case was combined with chemotherapy after surgical resection of abdominal lesions,and the other 4 cases were treated with combination chemotherapy alone.One of the patients relapsed after 4 years of chemotherapy,and then continued to receive 3 courses of chemotherapy in the original regimen.After the end of chemotherapy,they all achieved complete or partial remission,and 5 cases survived after follow-up.Conclution: The cause of Castleman's disease is unknown and lacks specific clinical manifestations.the clinical manifestations of MCD are diverse,which is easy to miss.Its diagnosis depends on the pathological biopsy.Therefore,the lymph node biopsy should be performed as soon as possible for suspected CD cases.MCD is usually associated with systemic symptoms.There is a lack of consensus on the best option for MCD treatment.Currently,the main treatment is combined chemotherapy.