Department of Pediatric Hemato-Oncology, Hospital Escuela-Universitario, Tegucigalpa, Honduras.
Department of Pediatric Oncology, Manuel de Jésus Rivera Hospital, Managua, Nicaragua.
Pediatr Blood Cancer. 2019 May;66(5):e27621. doi: 10.1002/pbc.27621. Epub 2019 Jan 24.
Treating B-non-Hodgkin lymphoma (B-NHL) in lower-income countries is challenging because of imprecise diagnosis, the increased risk of fatal toxicity associated with advanced disease at presentation, and limited supportive care.
Central American patients with newly diagnosed stage I or II B-NHL received a modified Berlin-Frankfurt-Münster (BFM) regimen including a prephase (prednisone, cyclophosphamide) followed by A/B/A courses (A: cytarabine, dexamethasone, etoposide, ifosfamide, methotrexate, and intrathecal therapy; B: cyclophosphamide, dexamethasone, doxorubicin, methotrexate, and intrathecal therapy). Those with stage III or IV NHL received additional courses (B/A/B), intensified for stage IV disease by additional vincristine and methotrexate doses. Patients in poor condition received a second prephase treatment before their chemotherapy courses.
Between March 2004 and June 2016, of 405 patients with B-NHL, 386 (109 females) were eligible for treatment. Immunohistochemistry was performed in 177 cases (47.4%) and characterized the disease as mature B-cell lymphoma. Stage distribution was as follows: I/II, 31 (8.1%); III, 252 (65.3%); IV, 93 (24.1%); 10 (2.6%) not available. The 3-year overall survival was 70% for the whole group (86% for stages I/II, 75% for stage III, 58% for stage IV). Events included death during induction (34 patients, 8.8%), relapse/progression (46, 11.9%), death in remission (9, 2.3%), second malignancy (1, 0.26%), and death of unknown cause (1, 0.26%). Twenty-three (6%) patients abandoned or refused therapy.
Approximately 70% of children with B-NHL from Central America experienced long-term, disease-free survival with a modified BFM schedule. Toxic death and relapse/resistant disease were the main reasons for treatment failure.
在低收入国家治疗 B 型非霍奇金淋巴瘤（B-NHL）具有挑战性，因为诊断不准确，在疾病进展时存在致命毒性风险增加，以及支持性护理有限。
新诊断为 I 期或 II 期 B-NHL 的中美洲患者接受了改良的柏林-法兰克福-明斯特（BFM）方案治疗，包括预阶段（泼尼松、环磷酰胺），然后是 A/B/A 疗程（A：阿糖胞苷、地塞米松、依托泊苷、异环磷酰胺、甲氨蝶呤和鞘内治疗；B：环磷酰胺、地塞米松、多柔比星、甲氨蝶呤和鞘内治疗）。那些患有 III 期或 IV 期 NHL 的患者接受了额外的疗程（B/A/B），IV 期疾病通过额外的长春新碱和甲氨蝶呤剂量进行强化。身体状况不佳的患者在接受化疗前接受了第二个预阶段治疗。
在 2004 年 3 月至 2016 年 6 月期间，405 名 B-NHL 患者中，有 386 名（109 名女性）符合治疗条件。177 例（47.4%）进行了免疫组织化学检查，将疾病特征化为成熟 B 细胞淋巴瘤。分期分布如下：I/II 期，31 例（8.1%）；III 期，252 例（65.3%）；IV 期，93 例（24.1%）；10 例（2.6%）不可用。全组 3 年总生存率为 70%（I/II 期为 86%，III 期为 75%，IV 期为 58%）。事件包括诱导期死亡（34 例，8.8%）、复发/进展（46 例，11.9%）、缓解期死亡（9 例，2.3%）、第二恶性肿瘤（1 例，0.26%）和原因不明的死亡（1 例，0.26%）。23 名（6%）患者放弃或拒绝治疗。
来自中美洲的约 70% B-NHL 儿童接受改良的 BFM 方案治疗后长期无病生存。毒性死亡和复发/耐药疾病是治疗失败的主要原因。
