Park Yeon Hee, Kim Won Seog, Kang Hye Jin, Na Im Il, Ryoo Baek-Yeol, Yang Sung Hyun, Lee Seung-Sook, Uhm Ji Eun, Kim Kihyun, Jung Chul Won, Park Keunchil, Ko Young H
Division of Hematology-Oncology, Department of Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological & Medical Sciences, 215-4, Gongneung-Dong, Nowon-Gu, 139-706, Seoul, South Korea.
Ann Hematol. 2006 May;85(5):285-90. doi: 10.1007/s00277-005-0050-8. Epub 2006 Mar 4.
Burkitt lymphoma/leukemia (BL) is a highly aggressive non-Hodgkin's lymphoma (NHL) often presenting in extranodal sites or as an acute leukemia. Because of the shared molecular and genetic features, the World Health Organization classification of lymphoid diseases recognizes the lymphomatous and leukemic phases of BL as a single entity: a mature B cell neoplasm, subtype Burkitt lymphoma/Burkitt cell leukemia. Because BL frequently appears in the stomach, we investigated the clinical presentation and outcome of gastric BL. We discovered 21 patients with gastric BL through a survey of our NHL registry, and retrospectively analyzed the distinct features of BL, including the St Jude/Murphy staging, other extranodal involvement, morphology and immunophenotype, response to treatment, and clinical outcome. The patients' median age was 48 years (range, 7-75), and the male-to-female ratio was 2.5. Stage 1 was found in five patients, stage 2 in five patients, and stage 4 in 11 patients. The stomach body and antrum were most frequently involved. All 21 patients were treated with systemic intensive chemotherapy, producing a 71% (15/21) rate of complete response (CR) to chemotherapy. Both the 2-year disease-free and overall survival rates were 55%. All ten patients in BL stages 1 and 2 showed outstanding outcomes, and nine of the ten patients exhibited CR and long-term survival. These data show that a high proportion of patients with gastric BL have a localized disease that is limited to stage 1 and 2, and that these localized BLs have outstanding outcomes. These findings suggest that these BLs could represent a distinct subtype that might have a different biology, which needs to be defined.
伯基特淋巴瘤/白血病（BL）是一种侵袭性很强的非霍奇金淋巴瘤（NHL），常发生于结外部位或表现为急性白血病。由于具有共同的分子和遗传特征，世界卫生组织淋巴系统疾病分类将BL的淋巴瘤期和白血病期视为一个单一实体：一种成熟B细胞肿瘤，亚型为伯基特淋巴瘤/伯基特细胞白血病。由于BL常出现在胃部，我们对胃BL的临床表现和预后进行了研究。通过对我们的NHL登记处进行调查，我们发现了21例胃BL患者，并回顾性分析了BL的不同特征，包括圣裘德/墨菲分期、其他结外受累情况、形态学和免疫表型、对治疗的反应以及临床预后。患者的中位年龄为48岁（范围7 - 75岁），男女比例为2.5。5例患者为1期，5例患者为2期，11例患者为4期。胃体和胃窦最常受累。所有21例患者均接受了全身强化化疗，化疗完全缓解（CR）率为71%（15/21）。2年无病生存率和总生存率均为55%。BL 1期和2期的所有10例患者均表现出良好的预后，其中10例患者中有9例达到CR并长期存活。这些数据表明，高比例的胃BL患者患有局限于1期和2期的局限性疾病，且这些局限性BL预后良好。这些发现提示这些BL可能代表一种具有不同生物学特性的独特亚型，有待进一步明确。
