| Objective To study the clinical features,treatment and prognosis of primary extranodal Hodgkin’s lymphoma(PEHL)of 11 cases and to analysis features of 75 cases in the PUBMED database who were diagnosed PEHL to explore the characteristics of these diseases.Methods We collected 11 cases who were treated in from the First Affiliated Hospital of Guangxi Medical University from January 2007 to December2017 and converged 75 cases who were diagnosed PEHL and obtained full-text case reports from PUBMED database in the same period.The clinical,pathological features and prognosis were analyzed by retrospective analysis.Result1.In 11 patients,the median age of onset was 35 years(12-58 years),mean age was 33.4±14.8 years.In the literature reviewed the median age of onset was46.5 years(3-88 years),mean age was 47.8±20.5 years.Overall the average Age is 45.9±20.4 years old,and the onset age is obviously bimodal,among which peak incidence is around 20-40 and 60 years old.Male to female ratio is approximately 1:1.15.2.The onset sites in 11 patients were most common in the lung(2/11),gastrointestinal tract(2/11),and thymus(2/11),Compared to the 75 case in the database common onset sites were in the gastrointestinal tract(17.3%),bone(17.3%),lung(14.7%),brain(13.3%).The clinical manifestations were nonspecific,with enlarged,compressed or dysfunctional organs in affected organs.Some patients(36.3% vs 37.3%)had B symptoms.11 patients were retrospectively diagnosed according to Ann Arbor and 4 were IE(36.3%)and 7 were IIE(63.7%).The IPS score was 6(54.5%)in the low-risk group and 5(45.5%)in the high-risk group.9 cases had low albumin(9/11),5 cases had anemia(5/11),and ESR increased in 4 cases(4/5).LDH increased in 3 cases(3/10),serum β2microglobulin increased in 2 cases(2/5),and CA125 increased in 3 cases(3/7).3.All 11 patients in our hospital and part of the database cases who had mentioned pathological data were more common with nodular sclerosis(45.5%vs 63.2%)and mixed cell type(45.5% vs 23.7%).All cases expressed CD30(100%).Most patients expressed CD15(63.6% vs 73.8%).NLPHL accounted for only 2 cases(5.3%)in 38 database reports.4.CT scans were performed in 10 patients in our hospital.9 patients were considered as malignant tumors.The detection rate was 90.0%.4 patients were diagnosed with PET/CT for definitive staging or evaluation of efficacy.5.11 patients in our hospital were treated with surgery,3 of them were treated with surgery alone,8 were treated with postoperative chemotherapy and3 were treated with surgery + chemotherapy + radiotherapy.1 patients were lost after surgery,and the 10 patients were followed up to February 1,2018.The longest follow-up period was 127 months.8 patients survived(80.0%),2 died(20.0%)and 2(20.0%)had relapsed.The five-year OS and DFS were 77.1% and72.0%.Conclusion1.PEHL is a group of diseases with obvious heterogeneity,with high incidence in two age groups of 20-40 and 60 years old.The incidence of PEHL is slightly higher than that of HL in women.2.PEHL is more common in bone,lung,and gastrointestinal tract.Clinical manifestations and pathological features are non-specific.Imaging examination has important implications in its diagnosis,staging,and differential diagnosis.CT can be used as the preferred imaging examination.3.The treatment strategy of PEHL is basically the same as that of lymph nodes H HL.The surgical diagnosis in PEHL is greater than the therapeutic effect.For patients with difficult puncture or endoscopic biopsy,the operation should be performed as soon as possible.Recommended conventional ABVD program for a full course of treatment.Radiotherapy can be used as a choice for combined treatment of focal lesions.4.The prognosis of PEHL was not significantly different from that of lymph nodes HL. |
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