| Objective:Analysis of 25 cases of Castleman disease (CD) clinical manifestations, laboratory features, with histopathological type, treatment, prognosis, improve the understanding of CD.Method: A retrospective analysis of the clinical and pathological data of patients with CD,which diagnosed by Histopathology during 2004-2014 in The First Affiliated Hospital of Guangxi Medical University.To summarize the clinical features of 25 patients, follow-up of all patients by telephone or outpatient, the data were statistically analyzed by SPSS 16.0 statistical software.Result:1.General information:included 25 cases of CD patients,16 males,9 females, with a median age of 49 years old (16-85 years old), with an average age of 47 years old.2. Clinical and pathological types:25 cases of 10 cases of CD patients in a unicentric Castleman disease, accounting for 40%, multicenter CD (MCD) 15 cases, accounting for 60%. Pathological types as hyaline vascular type 15 cases (60%), plasma cell type in 3 cases (12%), mixed cell type in 2 cases (8%), pathological type is unknown in 5 cases (20%).3. Clinical manifestations:clinical manifestations of 10 cases of UCD:single superficial lymphadenopathy in 3 cases (30%), single deep lymph node enlargement in 4 cases (40%), node involvement of peripheral lymphoid tissue in 3 cases (30%), all patients had no fever, night sweats, angular symptoms and hepatosplenomegaly; 15 cases of clinical manifestations of patients with MCD: 15 cases (100%) patients had multiple superficial lymph node enlargement,10 cases (66.7%) patients had multiple deep lymph nodes,5 cases (33.3%) patients associated with hepatosplenomegaly,9 cases (60%) patients accompanied by fever, sweating, weight loss, fatigue and other symptoms; with POEMS syndrome in 1 case with pulmonary damage in 3 cases (20%),1 case of paraneoplastic pemphigus,1 cases of rheumatoid arthritis, hemophagocytic comprehensive syndrome 1 case, nephrotic syndrome in 1 case.4.Laboratory examination:10 cases of UCD laboratory examinations of patients: 5 cases (50%) had mild anemia,1 cases elevated platelet count in (10%),3 cases (30%) appeared hypoproteinemia,4 cases patients improve erythrocyte sedimentation rate (ESR) examination,2 cases of patients with elevated erythrocyte sedimentation rate; 15 cases of MCD patients,6 cases (40%), elevated white blood cell count,8 cases (53.3%) patients had mild to moderate anemia,10 cases (66.7%) patients with elevated platelet count.7 cases (46.7%) hypoproteinemia,7 cases (46.7%) patients with elevated lactate dehydrogenase, 14 cases of patients with complete examination ESR,12 cases of patients with elevated ESR,12 cases underwent complete examination of C-reactive protein, 10 cases of elevated C-reactive protein in patients with,5 cases (30%) of autoantibodies in patients with positive,13 cases of patients improve immune globulin proteins examined, abnormalities in patients with immune globulin in 9 cases(immunoglobulin IgA, IgM, IgG increased or decreased),12 patients improve beta 2 microglobulin examination,10 cases of patients with beta 2 microglobulin increased.5.Treatment and curative effect:10 cases of UCD patients underwent operation treatment,10 patients reached CR.15 cases of MCD patients,4 patients gave up treatment.11 patients received treatment, including simple operation in 2 cases, 9 patients received systemic chemotherapy. Accept the surgical treatment of 2 cases occurred in patients with disease progression,9 patients who received chemotherapy, the complete remission in 1 cases, partial remission in 5 cases and disease progression in 3 cases.6. Prognosis:10 patients with UCD, with a median follow-up of 34 months (1-48 months), during the follow-up period, patients were maintained state of CR and the longest follow-up period in patients with maintain CR state has reached 48 months.15 cases of patients with MCD, the median follow-up time was 5 months (1-72 months),6 cases of patients after treatment to achieve CR/PR. During the follow-up period were not occurred disease progression, currently the longest survival time has reached 72 months; occurred in 5 patients with disease progression, death, median survival time was only 1 month; 4 cases of untreated patients,2 cases of death, survival time respectively 1 and 2 months; also two patients survived, the longest survival time has reached 5 months. Different clinical types, UCD 1 year,3 years,5 years survival rates were 100%, MCD 1 year,3 years,5 years survival rate were 56%, the differences in the survival curves between has statistical significance (P= 0.025); different pathological type. The 1,3 year survival rate, hyaline vascular type was 77%, plasma cell type was 60%, mixed cell type is 50%. Among different pathological types of survival rate difference no significant P=0.747. Cox regression models were applied to the age of this group of patients, the clinical typing, leukocyte count, lactic acid dehydrogenase (LDH), systemic symptoms, hepatosplenomegaly, albumin levels, treatment the prognostic factors were analyzed. The results show that each of the above factors on survival rate had no effect (P> 0.05).Conclusion:1.This study shows that:CD clinical type with multicentric CD, pathological type with hyaline vascular type was the most common.2.This study shows that:UCD clinical manifestations for asymptomatic local lymph node enlargement, not accompanied by fever, fatigue, weight loss, night sweats and hepatosplenomegaly, blood routine examination, blood biochemical examination no obvious abnormalities.3.This study shows that:Multicentric CD clinical main manifestation of lymph node enlargement and hepatosplenomegaly, often accompanied by fever, fatigue, night sweats, systemic symptoms, associated with lung injury and other organ damage, abnormal hematological and immunological examination, but no specificity.4.This study shows that:UCD with surgical treatment, curative effect, prognosis is good; MCD requires the use of systemic chemotherapy, the CHOP like chemotherapy, corticosteroids, interferon and thalidomide is sometimes effective; CHOP like scheme, glucocorticoid, interferon and thalidomide on pulmonary injury in patients with poor response, the mortality rate is high. The overall effect of MCD is poor, poor prognosis,need to try new treatments. |
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