医科院血液病医院侵袭性淋巴瘤诊疗策略和SCT经验分享.ppt

临床诊断：极易误诊 27例患者中仅7例本院初诊，其余20例在外院“确诊”，其中15例（75%）误诊； 9例误诊为CLL/SLL，其中8例在外院仅行骨髓穿刺形态或行活检而未做免疫组化诊为CLL，1例行脾切除后诊为SLL； 误诊为胃MALT淋巴瘤、淋巴母细胞淋巴瘤和弥漫大B细胞淋巴瘤各1例； 3例误诊为反应性淋巴结增生或淋巴结炎。 23例MCL的FISH结果 探针 阳性率 Del(13q) 8/24(37.5%) +12 1/23(4.3%) Del(11q) 4/23(17.4%) Del(17p) 10/23(43.5%) 总阳性率 13/23(56.5%) N 全阴 10(43.5%) 仅13q缺失 1 仅+12 1 仅ATM缺失 1 仅p53缺失 1 P53缺失伴13q- 5 P53与ATM同时缺失 1 P53/ATM/13q同时缺失 2 （中国医学科学院血液病医院2010) R-HyperCVAD/MA对所有患者总生存的影响 R-HyperCVAD/MA方案组的生存有优于其它方案的趋势！ （中国医学科学院血液病医院2010) R-HyperCVAD/MA显著改善 无p53缺失MCL患者的生存 （中国医学科学院血液病医院2010) P53缺失对生存的影响 B SE Wald df Sig. Exp(B) 95.0% CI for Exp(B) Lower Upper 肝肿大 .570 1.385 .169 1 .681 1.768 .117 26.689 P53缺失 4.095 1.486 7.593 1 .006 60.051 3.262 1105.367 IPI分组 -.229 1.201 .036 1 .849 .796 .076 8.370 年龄＞60岁 1.476 1.195 1.524 1 .217 4.373 .420 45.535 多因素预后分析 （中国医学科学院血液病医院2010) Our work:50 MCL患者的临床特征 characteristics Median age (range, year) 55.5(33-91) Sex (male) 38(76%) Median WBC (×109/L) 44.37(2.63-193.78) Median β2-MG (mg/L) 4.55(1.95-12.7) MIPI Low risk 12(24%) Medium risk 12(24%) High risk 26(52%) B symptom 18( 36%) splenomegaly 36(72%) hepatomegaly 3(6%) Comparison of the clinical and biological characteristics between MYC/BCL2 ± Clinical characterstics MYC+ MYC- p value BCL2+ BCL2- p value N 18 32 12 38 Median age (year) 55.0(33-71) 55.0(46-99) .606 52.0(33-91) 55.0(42-79) .481 Median WBC(×109/L) 35.43(2.63-168.89) 12.96(3.06-193.78) .052 14.32(3.91-84.88) 22.14(2.63-193.78) .413 Median β2MG(mg/L) 6.67(2.09-12.7) 3.76(1.95-6.76) .009 3.76(3.02-5.63) 4.63(1.95-12.7) .429 MIPI .036 .129 Low risk 1(5.6%) 11(34.4%) 7(58.3%) 31(81.6%) Medium/High risk 17(94.4%) 21(65.6%) 5(41.7%) 7(18.4%) B symptom 9(50%) 9(28.1%) .122 5(41.7%) 13(34.2%) .693 splenomegaly 3(16.7%) 0 .017 0 3(7.9) .315 hepatomegaly 14(77.8%) 22(68.8%) .495 8(66.7%) 28(73.7%) .637 Del(13q) 11(61.1%) 7(21.9%) .006 2(16.7%) 16(42.1%) .170 Del(11q) 6(33.3%) 3(9.4%) .034 1(8.3%) 8(21.1%) .425 Del(17p) 11(61.1%) 6(18.8%) .002 2(16.7%) 15(39.5%) .1