An effective therapy for both undifferentiated (including Burkitt's) lymphomas and lymphoblastic lymphomas in children and young adults.
An effective therapy for both undifferentiated (including Burkitt's) lymphomas and lymphoblastic lymphomas in children and young adults.
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对于儿童和年轻人的未分化（包括伯基特）淋巴瘤和淋巴母细胞淋巴瘤的有效治疗方法。
作者：
I. Magrath;C. Janus;B. Edwards;R. Spiegel;E. Jaffe;C. Berard;J. Miliauskas;K. Morris;R. Barnwell
我们使用单一的强化化疗方案来治疗患有弥漫性、侵袭性恶性淋巴瘤的年轻患者。在我们的研究中，淋巴瘤有两种主要的组织学类型：淋巴母细胞淋巴瘤，最常出现在纵隔肿瘤（64%），以及未分化淋巴瘤（主要是Burkitt淋巴瘤），主要发生在腹部（86%）。我们的目的是在包括31名儿童（2-16岁）和34名年轻人（17-35岁）的统一治疗患者组中检查预后的决定因素。骨髓广泛受累（肿瘤细胞替代超过50%）的患者被纳入研究。治疗主要包括4种药物组合（环磷酰胺、阿霉素、长春新碱和强的松）与42小时甲氨蝶呤输注交替进行，随后是白藜芦醇抢救，包括针对中枢神经系统（CNS）疾病的鞘内预防性治疗。局部或切除的未分化淋巴瘤患者接受6个治疗周期；所有其他患者接受15个周期。放射治疗只在特殊情况下使用。65例患者中有58例（89%）达到完全缓解：97%的儿童和82%的成人。估计3年生存率为60% (SE 6.4%)，中位随访时间为3年。与缓解持续时间和生存率相关的因素分析表明，转诊时骨髓受累和广泛疾病是不良预后变量。淋巴母细胞淋巴瘤患者和完全切除的未分化淋巴瘤患者预后最好（3年估计生存率分别为81% +/- 12%和94% +/- 6%）。广泛腹内未分化淋巴瘤（D期）患者预后最差（估计3年生存率为33% +/- 11%），但即使在该亚组中，骨髓受累也是一个不利因素（有和无骨髓受累的D期患者的估计生存率分别为14% +/- 13%和43% +/- 15%）。升高的尿酸和/或乳酸脱氢酶（LDH）也有预后意义，但主要反映状态，即疾病的程度。年龄对预后无显著影响。在未分化淋巴瘤亚组中，组织学（即伯基特淋巴瘤与非伯基特淋巴瘤）不具有预后意义。39%的循环中总白细胞计数低于1000 /立方毫米，17%的循环中出现伴有粒细胞减少的发热。50%的患者出现中度至重度口腔炎。（摘要删节为400字）
We have used a single intensive chemotherapy regimen in the treatment of young patients with diffuse, aggressive, malignant lymphomas. There were two major histologic types of lymphoma in our series: lymphoblastic lymphomas, which presented most often with mediastinal tumor (64%), and undifferentiated lymphomas (mostly Burkitt's lymphomas), which occurred predominantly in the abdomen (86%). Our objective was to examine the determinants of prognosis in a uniformly treated patient group that included 31 children (2-16 yr) and 34 young adults 17-35 yr). Patients with extensive bone marrow involvement (greater than 50% replacement by tumor cells) were included in the study. Treatment consisted essentially of a 4-drug combination (cytoxan, adriamycin, vincristine, and prednisone) alternating with a 42-hr methotrexate infusion, followed by leukovorin rescue, and included intrathecal prophylactic therapy against central nervous system (CNS) disease. Patients with localized or resected undifferentiated lymphoma received 6 therapy cycles; all other patients received 15 cycles. Radiation therapy was used only in exceptional circumstances. Fifty-eight of 65 patients (89%) achieved complete remission: 97% of children and 82% of adults. The estimated 3-yr survival was 60% (SE 6.4%) with a median follow-up of 3 yr. Analysis of factors associated with remission duration and survival indicated that bone marrow involvement at referral and extensive disease were poor prognostic variables. Patients with lymphoblastic lymphomas and patients with completely resected undifferentiated lymphomas had the best prognosis (81% +/- 12% and 94% +/- 6% estimated 3-yr survival, respectively). Patients with extensive intraabdominal undifferentiated lymphoma (stage D) had the worst prognosis (33% +/- 11% estimated 3 yr survival), but even in this subgroup, bone marrow involvement was an adverse factor (estimated survival in stage D patients with and without bone marrow involvement was 14% +/- 13% and 43% +/- 15%, respectively). Elevated uric acid and/or lactic dehydrogenase (LDH) were also of prognostic significance, but predominantly reflected state, i.e., extent of disease. Age did not significantly influence prognosis. In the undifferentiated lymphoma subgroup, histology (i.e., Burkitt's lymphoma versus non-Burkitt's lymphoma) was not of prognostic significance. Total white count was below 1,000/cu mm in 39% of cycles, and fever associated with granulocytopenia occurred in 17% of cycles. Stomatitis of moderate to severe extent occurred in 50% of cycles.(ABSTRACT TRUNCATED AT 400 WORDS)