Sandlund J T, Fonseca T, Leimig T, Verissimo L, Ribeiro R, Lira V, Berard C W, Sixbey J, Crist W M, Mao L, Chen G, Pui C H, Heim M, Pedrosa F
Department of Hematology-Oncology, St Jude Children's Research Hospital, the University of Tennessee, Memphis 38105, USA.
Leukemia. 1997 May;11(5):743-6. doi: 10.1038/sj.leu.2400609.
The purpose of this paper was to define the histologic distribution, clinical features, and treatment response of childhood non-Hodgkin lymphoma (NHL) in northeastern Brazil. We reviewed medical records and histopathologic studies of 98 children treated for NHL from 1980 to 1987 at a major pediatric cancer center in Recife, Brazil. Treatment outcome was evaluated in relation to tumor burden (stage and serum LDH) and type of therapy (LSA2L2 vs other multiagent chemotherapy). There was a striking predominance of the small noncleaved cell (Burkitt) subtype, which occurred in 92 of the 98 children and adolescents diagnosed with NHL. Subsequent analyses focused on these patients. The majority (n = 84) had advanced (stage III/IV) disease at diagnosis. The abdomen was the most common site of disease (84 cases); jaw involvement was rare (three cases). Five-year event-free survival (excluding treatment refusals) was significantly better for patients with limited vs advanced stage disease (75 +/- 14% vs 42 +/- 6%; P < 0.04). Elevated serum LDH (>500 U/l) was associated with a poorer outcome (P = 0.008). The type of chemotherapy did not affect EFS (P = 0.95). Only 39% of patients are long-term survivors, reflecting the high rate of septic deaths (25% of patients) and parental refusal/abandonment of therapy (10%). Epstein-Barr virus (EBV) was detected in tumor cells from eight of the 11 cases studied. In clinical presentation, these cases resemble sporadic Burkitt lymphoma, yet in their apparent responsiveness to LSA2L2 therapy and association with EBV, they do not. Childhood NHL in northeastern Brazil is predominantly of the Burkitt subtype, and is associated with clinical features that appear to distinguish it from the endemic and sporadic forms of this tumor. These cases may represent a third or intermediate subtype of Burkitt lymphoma.
本文旨在明确巴西东北部儿童非霍奇金淋巴瘤（NHL）的组织学分布、临床特征及治疗反应。我们回顾了1980年至1987年在巴西累西腓一家主要儿科癌症中心接受NHL治疗的98名儿童的病历和组织病理学研究。根据肿瘤负荷（分期和血清乳酸脱氢酶）及治疗类型（LSA2L2方案与其他多药化疗方案）评估治疗结果。小无裂细胞（伯基特）亚型显著居多，在98例诊断为NHL的儿童和青少年中有92例为此亚型。后续分析聚焦于这些患者。大多数患者（n = 84）诊断时已处于晚期（III/IV期）。腹部是最常见的病变部位（84例）；颌部受累罕见（3例）。局限期与晚期疾病患者的5年无事件生存率（不包括拒绝治疗者）有显著差异（75±14% 对42±6%；P < 0.04）。血清乳酸脱氢酶升高（>500 U/l）与较差的预后相关（P = 0.008）。化疗类型不影响无事件生存率（P = 0.95）。仅有39%的患者为长期幸存者，这反映出败血症死亡发生率高（25%的患者）以及家长拒绝/放弃治疗（10%）。在11例研究病例中，有8例肿瘤细胞检测到爱泼斯坦-巴尔病毒（EBV）。在临床表现上，这些病例类似散发性伯基特淋巴瘤，但在对LSA2L2治疗的明显反应性及与EBV的关联方面却并非如此。巴西东北部的儿童NHL主要为伯基特亚型，且具有一些临床特征，似乎使其有别于该肿瘤的地方性和散发性形式。这些病例可能代表伯基特淋巴瘤的第三种或中间亚型。
