目的:报道5例原发肾上腺弥漫大B细胞淋巴瘤(DLBCL)的临床以及病理特点,提高对原发肾上腺DLBCL的认识及诊治水平.方法:回顾性分析在我院诊治的5例原发肾上腺DLBCL患者的临床及病理资料.结果:5例原发肾上腺DLBCL,其中4例为老年男性;临床多以腰痛或腹痛为首发表现,5例患者均伴有B症状,4例伴有乳酸脱氢酶升高,1PI积分多为中高危;3例患者伴有肾上腺皮质功能不全,其中2例需要糖皮质激素替代治疗;影像学均为双侧肾上腺肿块,超声或CT引导下穿刺病理确诊;病理4例呈现双表达DLBCL,活化B细胞来源;未见骨髓受累患者.原发肾上腺DLBCL采用包含利妥昔单抗的联合化疗疗效较理想,但停药易复发,应用来那度胺或沙利度胺维持治疗可能可以减少复发;治疗需要兼顾中枢神经系统淋巴瘤的预防,包括大剂量甲氨蝶呤或来那度胺预防.结论:本研究结果提示发现双侧肾上腺占位需要警惕原发肾上腺淋巴瘤,确诊需要活检病理证实;原发肾上腺DLBCL治疗建议含有利妥昔单抗的联合化疗,同时给予中枢淋巴瘤预防,联合化疗结束后建议维持治疗1～2年有助于减少复发.

Objective To evaluate the feasibility of using comprehensive geriatric assessment (CGA) in estimating if standard dose treatment is fit for the elderly patients with diffuse large B cell lymphoma.Methods.Comprehensive geriatric assessments including three assessments of activity of daily living,instrumental activity of daily living and comorbidity scoring according to Cumulative Illness Rating Score for Geriatrics were adopted to assess if standard dose treatment is fit for the elderly patients in our prospective study.Thirty seven patients with diffuse large B cell lymphoma,aged ＞70 years were enrolled in the study,and grouped into fit,unfit and frail groups according to comprehensive geriatric assessment scoring and their age.The treatment protocolswere not determined by comprehensive geriatric assessment scores,but by clinical judgments made by clinicians based on their clinical experience and disease features.The clinically effective response and overall survival (OS) were analyzed in the three groups.Results According to CGA scores,patients were grouped into "fit" [21 cases (56.8％)],"unfit" [7 (18.9％)] and "frail" [9 (24.3％)].37 cases received 213 courses of treatment at average 5.76 courses per case.The overall response (complete / partial remission) rates were [85.7％(18/21) vs.28.6％ (2/7) vs.44.4％ (4/9),x2=9.69,P=0.008] and median survival times were (44 months vs.10 months vs.9 months;x2 =7.03,P=0.03) among "fit","unfit" and "frail" groups with statistically significant differences.Total effective rate (achieving all clinical targets) in "fit" group of 21 cases were 100 ％ (12/12)with receiving standard dose therapy,and 66.7％ of(6/9)with low dose therapy(P=0.06).Overall response rate(total/partial remission) [85.7％(18/21) vs.28.6％(2/7) vs.44.4％(4/9),x2=9.69,P=0.008] and median survival (44 months vs.10 months vs.9 months;x2 =7.03,P=0.03) among"fit","unfit" and "frail" groups.In "fit" group,the two-year overall survival was higher in patients receiving standard dose treatment than receivingpalliativetreatment,with statistical significance [83.3 ％ (10/12) vs.33.3 ％ (3/9),P =0.032],without significant hematologic toxicity observed between the subgroups.Conclusions Comprehensive geriatric assessment can identify if elderly patients diffuse large B cell lymphoma can acquire a satisfactory curative effect from a standard dose treatment ofimmunochemotherapy.

目的减少临床中恶性肿瘤表现为血栓性血小板减少性紫癜(TTP)时的误诊、漏诊。方法报道1例恶性肿瘤骨髓转移表现为TTP的患者, 并复习相关文献。结果该例患者表现为TTP并同时伴有明显的呼吸困难及骨痛, 一般情况差, 无法耐受血浆置换及放化疗。予血浆输注、甲泼尼龙静脉滴注, 但效果不理想, 入院15 d患者死亡。结论恶性肿瘤表现为TTP时极易误诊、漏诊, 需警惕恶性肿瘤的存在, 应尽早行骨髓活组织检查, 及时选择适当的治疗方式。

Objective To explore the prognostic factors and treatment regimens of diffuse large B-cell lymphoma (DLBCL) in elderly patients.Methods The data of characteristics,treatment regimens and outcomes were collected in elderly patients aged 70 years and over,with newly diagnosed DLBCL from our hospital in the last decade.Then the factors influencing therapeutic response,overall survival and progression-free survival were analyzed.Results A total of 49 patients aged 70 to 92 years (median 76 years) were enrolled in this study.Among them,18 patients were aged 80 years and older;the other 31 patients were aged less than 80 years.The treatment regimen was based on Rituximab (R),Cyclophosphamide (C),Adriamycin (H),Vincristine (O),Prednisone (P) (R-CHOP).For the patients with worse performance status,or≥80 years,50％ 75％ of standard dose of CHOP plus 100％ of Rituximab (R) dose were as starting dose.The complete remission rate was 46.9％ and partial remission rate was 18.4％,the overall effective rate was 65.3％.The 30 cases were in deaths.The median overall survival period was 25 months.The overall survival prolongation was associated with Ann Arbor staging Ⅰ-Ⅱ,serum lactate dehydrogenase less than 245 U/L,age less than 80 years,IPI≤2 and completing 6 cycles of R-CHOP.And multivariate regression analysis showed that serum lactate dehydrogenase less than 245 U/L and 6 cycles of R-CHOP were the independent prognostic factors.Among patients who completed 6 cycles of R-CHOP treatment regimen,there were 7 patients aged over 80 years (38.9％) and 17 patients aged less than 80 years (54.8％).There were no significant differences between different age groups in overall survival (32 months vs.34 months) and in progression free survival (32 months vs.32 months).Conclusions Reduced treatment intensity so as to complete the 6 cycles of R-CHOP treatment regimen could improve the outcomes of older patients who could not tolerance the dose of standard treatment regimen.And 6 cycles of R-CHOP treatment regimen are the independent prognostic factor for survival.

目的探索以硼替佐米为基础的联合化疗序贯自体外周血干细胞移植治疗原发性浆细胞白血病(pPCL)的效果。方法报道1例经硼替佐米为基础的联合化疗序贯自体外周血干细胞移植治疗pPCL,并进行相关文献复习。结果该例患者经硼替佐米为基础的联合化疗达到非常好的部分缓解(VGPR),进行自体外周血干细胞移植后达到完全缓解(CR),无进展生存达33个月。结论以硼替佐米为基础的联合化疗及自体造血干细胞移植可能会改善pPCL患者的预后,减少复发。

Objective To explore the clinical characteristics and prognostic value of monosomal karyotype（MK）patients in adult acute myeloid leukemia（AML）.Methods We retrospectively studied 45 patients of MK+in newly-diagnosed adult AML in our center from Oct 2000 to Dec2012.Clinical characteristics,cytogenetic data and prognostic features were analyzed in the cohort of MK+patients.Results MK was found in 45 patients（19.0%）

目的:分析老年急性髓细胞白血病(AML)的临床及生物学特点,寻求有效治疗方法。方法:回顾性分析62例60岁以上AML患者的临床资料,包括临床特征、染色体、免疫分型及化疗效果。结果:完全缓解(CR)率27.7%,总有效率44.7%。诱导期死亡率32.7%。伴MDS病史者占27.4%,其CR率低于无MDS病史者(6.7%vs37.5%,P<0.05)。伴淋系抗原表达者占28.2%,其CR率低于仅有髓系抗原表达者(0vs37.5%,P<0.05)。CD34+者占71.8%,其CR率低于CD34-者(16.0%vs62.5%,P<0.05)。结论:老年AML总体缓解率低,死亡率高,在临床上有其特殊性,治疗上更强调个体化。

Objective To explore the clinical and biological characteristics and treatment effect of acute myeloid leukemia (AML) in elderly patients. Methods The clinical data of 62 patients over 60 years old with AML were retrospectively analyzed, and they were compared with those of 60 younger adult patients (18-59 years old) at the same period. Results In elderly patients, the complete remission (CR) rate was 27. 7% and the overall effective rate was 44.7%, which were lower than those of younger adult patients (74.1% and 87.9 %, 1-espectively, P＜0.01). The early death rate was 19.0% and the mortality rate during the first two induction chemotherapy was 29. 3% in the elderly, which were higher than those of younger adult patients (3. 3% and 6.7%, 1-espectively,P＜0.01). The 27.4% of elderly patients were diagnosed as myelodysplastic syndrome (MDS)-transformed AML, which were more than that of younger adult patients (10.0%, P＜0.05), and they had lower CR rate than those without MDS (P＜0. 05). The 28.2% of elderly patients had lymphoid antigen positive AML and 71.8% of patients showed CD34+ which were higher than those of younger adult patients (8. 1% and 48.6%, respectively, P＜0. 05), and they had lower CR rate than those of lymphoid antigen negative and those of CD34- AML (P＜0. 05). Elderly patients had less favorable and more unfavorable karyotypes than younger adult patients (45.7 % and 15.4 %, P＜0. 05). Conclusions The elderly patients with AML have more unfavorable prognostic factors than younger adult patients. They have lower CR rate and higher mortality rate. There are many specialties in elderly patients and the treatment strategy should be made more individually.

Objectives To evaluate the efficacy of rituximab combined with chemotherapy in the treatment of diffuse large B-cell lymphoma (DLBCL) and the relationship of clinical prognosis with the International Prognostic Index (IPI) by the using rituximab in autologous peripheral stem cell transplantation (APBSCT) for the patients of DLBCL. Methods 21 patients with DLBCL, 11 patients of them were at IPI low risk, and 3 patients were IPI at low intermediate risk, 3 patients were at IPI high intermediate risk, 4patients IPI high risk. Rituximab combined with CHOP regimen (cyclophosphamide, adriamycin, vincfistine and prednisone) was given for 4～8 courses. 5 patients received APBSCT. The mobilizing regimen was rituximab combined with cyclophosphamide(CTX) and etoposide(VP16). The conditioning regimen were CBV(CTX combined with VP16 and carmustine). Results In 21 patients, the complete response rate was 61.9 %,with overall response rate 90.5 %. 2-year progression free survival was (69.74±10.43)%. 2-year overall survival was (84.44:1:8.35) %. The complete response rate was 92.9 % and overall response rate was 100 % in the patients IPI≤2. The overall response rate was 71.4 % in the patients with IPI≥3. The complete response rate was higher in the patients with IPI≤ 2 (P＜0.01). The amount of mononuclear cells (M NC) in harvest were 7.34 (4.6～8.53)×108/kg. The CD+34 cells in harvest were 8.82 (2.1～10.34)×1O6/kg. The mean time of neutrephil recovering to 0.5×109/L after APBSCT was +9 day. The mean time of platelet recovering to 20×109/L after APBSCT was +12 day. The major adverse reaction were infusion related response (14.3 %) and hematological toxieities. Conclusion The efficacy of rituximab combined with chemotherapy in the treatment of DLBCL is effective, The complete response rate was higher in the patients with IPI≤2 than in the patients with IPI≥3.Using rituximab in mobilizing regimen, all patients had harvested enough CD+34 cells. Rituximab given at +1day did not affect the hematopoiesis reconstruction.

目的 观察中大剂量阿糖胞苷作为急性髓系白血病(AML)缓解后强化治疗方案的临床疗效及其对自体外周造血干细胞采集及造血重建的影响;观察染色体核型分析结果 与临床预后的关系.方法 15例AML患者缓解后予中大剂量阿糖胞苷强化治疗;此后有3例患者接受异基因造血干细胞移植(allo-SCT),7例接受自体外周造血干细胞移植(APBSCT).结果 2例在CR1期行非血缘allo-SCT,日前均无病生存,1例在复发期行单倍型allo-SCT,在+110天复发;7例行APBSCT的患者中3例无病生存,另外4例复发;未行移植的5例患者均死亡.7例接受APBSCT的患者采集的中位MNC 7.33×108/kg,中位CD+34细胞6.9×106/kg,中性粒细胞>0.5×109/L的中位时间+11天,血小板>20×109/L的中位时间+13大.具有预后良好核型的患者3例,2例无病生存;具有预后不良核型的患者4例,2例行allo-SCT,1例行APBSCT,目前均无病生存,另外1例死亡;具有预后中等核型的患者共6例,其中1例联合APBSCT,目前无病生存,其余患者均死亡.结论 中大剂量阿糖胞苷不联合造血干细胞移植作为AML缓解后强化治疗患者复发率较高,具有预后中等和不良染色体核型的患者行allo-SCT可能改善预后;采用中大剂量阿糖胞苷巩固强化治疗的患者均能采集到足够的造血干细胞并能造血重建.

目的:通过对变异型毛细胞白血病(HCL-V)的临床及形态学特征探讨提高对诊断HCL-V的认识.方法:报告1例HCL-V的临床及形态学、细胞遗传学、免疫学特点,结合文献进行分析讨论.结果:患者间断发热2周入院,入院前曾在我院急诊科用希刻劳抗感染治疗,无明显效果,经骨髓细胞形态学、组织化学、相差显微镜、电镜、骨髓活检及免疫组化检查诊断为HCL-V.结论:明确诊断HCL-V仅依赖于骨髓细胞形态学较为困难,必须结合患者临床特征以及相关检查,才能明确诊断,进而得到及早治疗.

目的对比改良VAD(AVD)与非VAD方案治疗多发性骨髓瘤(MM)的疗效及毒副作用.方法41例MM患者,分为A、B两组.A组21例,每例应用AVD方案5个疗程[阿霉素9mg/(m2/d),西艾克0.5mg/d,二者均为快速静脉输注,d1-4;地塞米松40mg,POd1-4,d7-12,d17-20;28～35天为1个疗程],共105个疗程.B组20例,每例应用MP或M2方案5个疗程,共100个疗程.监测血常规,肝、肾功能、生化指标及毒副反应,每疗程结束复查骨髓,X线,免疫球蛋白定量,评价生理状态等.结果A组治疗3个疗程后,CR1例,PR12例,有效率61.9%;5个疗程后,CR2例,PR12例,总有效率66.7%.B组3个疗程后,PR5例,有效率25%;5个疗程后,PR 7例,总有效率35%.A组有效率明显高于B组(P＜0.05).毒副反应比较:主要为感染及WBC减少.A组感染6例,占28.6%(6/21);WBG减少(＜2.5×109/L)22个疗程,占总疗程的21%(22/105).B组感染5例(25%)(5/20),WBC减少6个疗程,占总疗程的6%(6/100).A组WBC减少较B组明显(P＜0.05),感染发生率较高,但差异无显著性(P＞0.05).结论快速静脉输注阿霉素,西艾克改良VAD方案,为一疗效佳,见效快,病人易于接受的治疗方案.

尽管淋巴瘤的治疗已经有了很多进展,但是微小残留病灶仍然很难被现行放疗、化疗方案彻底消灭.目前,应用基因手段提高淋巴瘤的疗效已取得了很大进展,尤其是应用淋巴细胞表面独特型抗原制备的DNA疫苗治疗淋巴瘤,现已进入临床试验阶段.