Dembowska-Baginska Bozenna, Perek Danuta, Brozyna Agnieszka, Wakulinska Anna, Olczak-Kowalczyk Dorota, Gladkowska-Dura Malgorzata, Grajkowska Wieslawa, Chrzanowska Krystyna H
Department of Pediatric Oncology, The Children's Memorial Health Institute, Warsaw, Poland.
Pediatr Blood Cancer. 2009 Feb;52(2):186-90. doi: 10.1002/pbc.21789.
Due to small number of patients with Nijmegen Breakage Syndrome (NBS) and Non-Hodgkin lymphoma (NHL) experience in their treatment is limited.
Since 1996, 17 patients with a median age of 9.5 years who had NBS, were treated for NHL. NHL type, stage, chemotherapy, dose modifications, chemotherapy delays, response to chemotherapy, toxicity, outcome and correlation of drug reduction with response to treatment and outcome were analyzed.
Nine patients had TNHL, eight BNHL. TNHL patients received BFM and BNHL LMB type protocols. Doses of cytostatics were reduced in the first chemotherapy courses. Further modifications depended on severity of complications. None of the patients complied with timing of chemotherapy. Complete remissions after induction were achieved in 8 of 9 TNHL and 3 out 8 of BNHL patients. All patients experienced grade 4 toxicities. Two patients died from complications. Six of 17 patients are alive. All received more than 80% of recommended doses of chemotherapy. No differences in the type, number of responses or grade 3 and 4 toxicities between patients receiving less or more than 80% of drug doses were observed. Treatment related deaths concerned patients who received less than 80% of drug doses.
Patients with NBS develop both T and B cell lymphomas. Treatment outcome is poor and might be improved by administering over 80% of drug doses. Although toxicity often depends upon drug doses, our patients experienced equal grade 3 and 4 toxicities whether they received more or less than 80% of the chemotherapeutic agents.
由于尼美根断裂综合征（NBS）患者数量较少，其在非霍奇金淋巴瘤（NHL）治疗方面的经验有限。
自1996年起，对17例中位年龄为9.5岁的NBS患者进行NHL治疗。分析了NHL类型、分期、化疗、剂量调整、化疗延迟、化疗反应、毒性、结局以及药物减量与治疗反应和结局的相关性。
9例为T细胞型非霍奇金淋巴瘤（TNHL），8例为B细胞型非霍奇金淋巴瘤（BNHL）。TNHL患者接受BFM方案，BNHL患者接受LMB型方案。在首个化疗疗程中细胞毒性药物剂量降低。进一步的调整取决于并发症的严重程度。没有患者遵守化疗时间。9例TNHL患者中有8例在诱导后达到完全缓解，8例BNHL患者中有3例达到完全缓解。所有患者均出现4级毒性。2例患者死于并发症。17例患者中有6例存活。所有存活患者接受的化疗剂量均超过推荐剂量的80%。在接受药物剂量低于或高于80%的患者之间，未观察到反应类型、反应数量或3级和4级毒性的差异。与治疗相关的死亡涉及接受药物剂量低于80%的患者。
NBS患者会发生T细胞和B细胞淋巴瘤。治疗结局较差，给予超过药物剂量80%的化疗可能会改善结局。尽管毒性通常取决于药物剂量，但我们的患者无论接受的化疗药物剂量是多于还是少于80%，均出现了相同程度的3级和4级毒性。
