Sporadic Burkitt Lymphoma in Adults; Clinico-pathologic Characteristics and Immunohistochemical Study

PURPOSE: The purpose of this study was to evaluate the clinico-pathologic features, immunohistochemical stains and treatment outcome of patients with sporadic Burkitt lymphoma (BL) in adults. METHODS: A survey of 1,422 NHL patients who had been diagnosed at Korea Institute of Radiological & Medical Sciences between 1993 and 2003 identified 26 patients with BL (1.8%). All these cases were reviewed by experienced hemato-pathologist. The clinical data of these 26 patients were analyzed retrospectively. We did immunohistochemical stains for immunophenotype determination and EBER-1 in situ hybridization (ISH) for evaluatin of relationship between EBV infection and BL. RESULTS: Among the 26 patients, there were 14 men and 12 women (median age: 40.5, range: 16-67 years). All patients were HIV-negative. The ileo-cecal region represented the most frequent site of involvement (42.3%). Bulky disease was 26.9%(7/26). Bone marrow and Cerebrospinal fluid involvement were 30.8%(8/26) and 11.5%(3/26), respectively. Twenty-five patients were treated with chemotherapy. Chemotherapy regimens included CHOMP, CODOX/IVAC, and high intensity chemotherapy with brief duration. Among these, three patients were treated with high dose chemotherapy(HDT) with atuo-transplantation(ASCT). Response rate to initial chemotherapy was 81.8%(CR 63.6%, PR 18.2%). We experienced treatment-related deaths in 3 patients. The median overall survival and event-free survival were 15.9months(95% C.I.: 0.4-38.2) and 6.0 months (95% C.I.: 3.8-8.2), respectively. Characteristic immunophenotype illustrated by immunohistochemistry was CD20(+), CD10(+), CD3(-), Bcl-2(-), CD138(-), Ki67(+,100%). EBER-1 in situ hybridization(ISH) showed positive results at 25% of studied patients. CONCLUSION: We demonstrated that BL is a rare disease among adults in Non-Hodgkin lymphoma (NHL) and a highly aggressive malignancy in spite of the initial good response. However, patients with complete reponse after aggressive treatment have good prognosis with long-term survival. Further investigation to identify biologic feature and treatment modality with more patients should be continued.

Author dc:creator

• 윤, 성민

Contributors dc:contributor

• 최, 진혁
• 대학원 의학과
• 200024210

• 성인
• 산발형
• 버킷 림프종
• 면역조직화학적 염색
• EBV
• Adult
• sporadic
• Burkitt lymphoma
• immunohistochemical stains

Language dc:language

ko

Identifier

http://dcoll.ajou.ac.kr:9080/dcollection/jsp/common/DcLoOrgPer.jsp?sItemId=000000000438
000000000438

OAI identifier oai:identifier

oai:repository.ajou.ac.kr:201003/2296