Zvonkov E E, Kremenetskaia A M, Kravchenko S K, Makhinia V A, Kaplanskaia I B, Obukhova T N, Samoĭlova R S, Shevelev A A, Magomedova A U, Bariakh E A, Krasil'nikova B B, Gubkin A V, Iliushkina E A, Mar'in D S, Morozova A K, Kulikov S M, Gemdzhian E G, Vorob'ev A I
Ter Arkh. 2008;80(7):18-26.
To compare efficacy and toxicity of conservative therapy (different programs of polychemotherapy) of gastric lymphosarcoma conducted for the last 10 years in Hematological Research Center of the Russian Academy of Medical Sciences.
The study included 63 patients (40 females and 23 males aged 14 to 78 years, mean age 49 years) with primary diagnosis of gastric lymphosarcoma (GL). Of them, 56 (89%) patients had diffuse large B-cell lymphosarcoma (DLBCL) and 7 (11%) had gastric Berkitt's lymphoma (BL). Only detection of t(8;14) with rearrangement of c-myc gene provided accurate diagnosis of gastric BL. By the treatment DLBCL patients were divided into two groups: 44 patients of group 1 received polychemotherapy (PCT) according to CHOP scheme or in combination with radiotherapy and surgical treatment; 12 patients of group 2 were treated according to modified program mNHL-BFM-90, without surgical or radiation treatment. Of 7 patients with gastric BL 5 patients received treatment according to a modified program mNHL-BFM-90 and 2 patients were given CHOP because of DLBCL misdiagnosis without cytogenetic detection of t(8;14).
Overall survival in group 1 was 73% in mean follow-up 61 months. The survival depended only on initial factors of poor prognosis (PPF): tumor size over 10 cm, Ann-Arbor stage higher than IE, B-symptoms, elevated level of LDH. Overall survival of 18 gastric DLBCL patients without PPF reached 94%, of 26 patients with PPF - 60%. Lethality due to side effects was 4% (2 patients), primary resistance was 14% (6 patients), recurrence arose in 9% (4 patients). Overall survival in group 2 was 100% in mean remission duration 18 months, was unrelated to PPF (10 of 12 patients) but correlated with high toxicity. 5 BL patients treated with a modified mNHL-BFM-90 program achieved remission (a mean follow-up at present is 1 to 50 months, mean 24 months). 2 BL patients treated with CHOP died for a year.
Gastric lymphosarcomas are sensitive to chemotherapy, thereby PCT only is effective in most patients. PPF in gastric DLBCL were responsible for poor outcome in 40% patients in CHOP treatment. The modified program mNHL-BFM-90 can produce up to 100% complete long-term remissions in therapy of gastric lymphosarcoma in adults both in BL and DLBCL patients. A cytogenetic examination of c-myc gene rearrangement is obligatory before initiation of PCT of gastric lymphosarcoma.
比较俄罗斯医学科学院血液学研究中心过去10年对胃淋巴肉瘤进行的保守治疗（不同方案的多药化疗）的疗效和毒性。
该研究纳入63例患者（40例女性和23例男性，年龄14至78岁，平均年龄49岁），初步诊断为胃淋巴肉瘤（GL）。其中，56例（89%）患者为弥漫性大B细胞淋巴肉瘤（DLBCL），7例（11%）为胃伯基特淋巴瘤（BL）。只有检测到t(8;14)伴c-myc基因重排才能准确诊断胃BL。根据治疗方法，DLBCL患者分为两组：第1组44例患者接受根据CHOP方案的多药化疗（PCT）或联合放疗和手术治疗；第2组12例患者根据改良方案mNHL-BFM-90进行治疗，未进行手术或放疗。7例胃BL患者中，5例根据改良方案mNHL-BFM-90接受治疗，2例因DLBCL误诊且未进行t(8;14)细胞遗传学检测而接受CHOP治疗。
第1组的总生存率在平均随访61个月时为73%。生存率仅取决于预后不良的初始因素（PPF）：肿瘤大小超过10 cm、Ann-Arbor分期高于IE期、B症状、乳酸脱氢酶水平升高。18例无PPF的胃DLBCL患者的总生存率达到94%，26例有PPF的患者为60%。因副作用导致的死亡率为4%（2例患者），原发性耐药为14%（6例患者），复发率为9%（4例患者）。第2组的总生存率在平均缓解期18个月时为100%，与PPF无关（12例患者中的10例），但与高毒性相关。5例接受改良mNHL-BFM-90方案治疗的BL患者实现缓解（目前平均随访1至50个月，平均24个月）。2例接受CHOP治疗的BL患者在一年内死亡。
胃淋巴肉瘤对化疗敏感，因此大多数患者仅PCT有效。胃DLBCL中的PPF导致CHOP治疗中40%的患者预后不良。改良方案mNHL-BFM-90在治疗成人胃淋巴肉瘤（包括BL和DLBCL患者）时可产生高达100%的完全长期缓解。在开始胃淋巴肉瘤的PCT之前，必须进行c-myc基因重排的细胞遗传学检查。
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