Zhao Yz

[Clinical outcome of autologous stem cell transplantation as first-line treatment in 30 patients with high risk lymphoblastic lymphoma].

OBJECTIVE To investigate the treatment outcomes of autologous stem cell transplantation (ASCT) as first-line treatment in patients with high risk lymphoblastic lymphoma (LBL) and compare the effect of different induction regimen on prognosis. METHODS Thirty LBL patients in complete remission received ASCT from 1996 to 2012 in our hospital were retrospectively analyzed. RESULTS (1)Of the 30 patients, 25 were T-LBL and 5 B-LBL with a median age of 19(7-53) years old. Ratio of male to female is 23:7. Fourteen (46.7%) patients presented with bulky mediastinal masses and 15(50.0%) with bone marrow involvement. The distribution of stages was 2(6.7%), 5(16.7%) and 23 (76.6%)patients with stages II, III, and IV, respectively. The distribution according to age-adjusted international prognostic index (aaIPI) was 5(16.7%) patients in 1 score, 14(46.6%) in 2 scores and 11(36.7%) in 3 scores. (2)At a median follow-up of 32(range, 10-171) months, 17 patients were alive and 13 relapsed and died from LBL after ASCT. The estimated 5-year probability of DFS and OS was (50.4±10.7) % and (53.9 ±10.2)% for all the patients. (3)According to the treatment regimens before ASCT, the patients were divided into NHL-type group (n=12) and ALL-type group (n=18). In NHL-type group, 9 patients relapsed and died, the estimated 5-year probability of DFS and OS was (22.2 ±12.8) % and (33.3 ±13.6) %, respectively. Median DFS and OS time were 24 months and 36 months. In ALL-type group, 4 patients relapsed and died from lymphoma, the estimated 5-year probability of DFS and OS was (77.8 ± 9.8) % and (77.8 ± 9.8) %, respectively. Median DFS and OS time were not reached. For DFS and OS, ALL-type group were better than that of NHL-type group and the difference was significant (P=0.022 and P=0.049). CONCLUSION The results showed that complete remission with intensive first-line ALL-type regimens and followed by ASCT consolidation may significantly improve long-term outcome for high risk LBL patients.

[Clinical analysis of multiple myeloma patients with bone-related extramedullary disease: a longitudinal study on 834 consecutive patients in a single center of China].

目的 探讨多发性骨髓瘤（MM）患者中单纯骨旁髓外病变（bEMD）的发生率、临床特征、预后意义，以及与严格的髓外病变（sEMD）的相关性。 方法 对1993年1月至2013年3月收治的834例连续性初诊MM患者的临床资料进行回顾性分析。 结果 ①834患者中初诊伴bEMD者32例（3.8%），伴sEMD者40例（4.8%）。与伴sEMD者比较，伴bEMD者乳酸脱氢酶（LDH）水平显著降低（180.9 U/L对299.2 U/L，P=0.034），治疗后的总体反应率（ORR）明显增高（95.7％对66.7%，P=0.009）；与无EMD者比较，其LDH水平和ORR差异均无统计学意义（P值均>0.05）。②未接受自体造血干细胞移植（ASCT）的患者中，初诊时伴sEMD（39例）、bEMD(24例）及无EMD（671例）者的中位总体生存（OS）时间分别为14.0、37.5和38.0个月，中位疾病进展（TTP）时间分别为11.5、27.0和22.0个月，与伴sEMD者比较，后两组患者的OS、TTP时间均明显延长（P值均<0.05），但后两组间比较，差异均无统计学意义（P值均>0.05）。③随访过程中，bEMD的发生率为0.5%，发生sEMD、bEMD和无EMD者的中位OS时间分别为26.0、17.0和40.0个月，中位TTP时间分别为13.0、11.0和25.0个月，发生bEMD与其他两组患者间差异均无统计学意义（P值均>0.05）。 结论 伴bEMD的MM患者具有不同于伴sEMD者的临床特征，其预后明显好于后者，与无EMD者比较预后相近，提示单纯伴bEMD对MM患者预后无明显不良影响。OBJECTIVE To analyse the incidence, clinical features, prognosis of bone-related extramedullary disease （bEMD） and its relationship with strict EMD (sEMD) in MM patients. METHODS The records of 834 consecutive newly diagnosed patients with MM in our hospital between 1993 and 2013 were retrospectively reviewed. RESULTS ①Among 834 patients at diagnosis, 32 cases (3.8%) showed bEMD, and 40 cases (4.8%) showed sEMD. Patients with bEMD at presentation showed significant lower level of lactate dehydrogenase (180.9 U/L vs 299.2 U/L, P=0.034) and higher overall response rate (ORR) (95.7% vs 66.7%, P=0.009) compared with sEMD patients. While the above two parameters were comparable between patients with bEMD and without EMD. ②As to the prognosis of patients without autologous hematopoietic stem cell transplantation (auto-HSCT), the overall survival (OS) of patients with sEMD, bEMD and without EMD was 14.0, 37.5, and 38.0 months, respectively. The time to progression (TTP) of the three groups was 11.5, 27.0, and 22.0 months, respectively. Compared to the patients with sEMD, the outcomes (both OS and TTP) of the other two groups was significantly better (P<0.05). Patients with bEMD at presentation was comparable to the patients without EMD, but the two groups were better than the patients with sEMD. ③The incidence of bEMD during follow-up was 0.5%. The OS of patients with sEMD, bEMD and without EMD during follow-up was 26.0, 17.0, and 40.0 months, respectively. The TTP of the three groups was 13.0, 11.0, and 25.0 months, respectively. The outcomes (both OS and TTP) of patients with bEMD at relapse/progression showed no significant difference as compared with the other two groups (P>0.05). CONCLUSION The clinical features of MM patients with bEMD are different from the patients with sEMD. Outcomes of this population is significantly better than the latter, and is comparable to the patients without EMD. It suggests that bEMD alone has no negative prognostic significance in MM patients.

[The characteristics of 62 cases of CD20-positive multiple myeloma].

目的 分析CD20阳性多发性骨髓瘤（MM）患者的临床和实验室检查特征。 方法 回顾性分析62例CD20阳性初诊MM患者的临床及实验室检查资料，并与CD20阴性患者进行比较。 结果 ①采用流式细胞术进行CD20分析的465例初诊MM患者中，CD20阳性者62例（13.3%）。②CD20阳性与阴性组患者比较，其性别、年龄、Durie-Salmon分期、ISS分期、肾功能损害、LDH水平、血小板计数及免疫球蛋白类型差异均无统计学意义（P值均>0.05）。③CD20阳性组患者血红蛋白水平（74.5 g/L对83.5 g/L，P=0.021）、髓外浸润发生率（3.5%对13.7%，P=0.029）、CD56阳性细胞率（36.7%对68.8%，P=0.000）、t（4;14）发生率（2.4%对24.0%，P=0.001）明显低于CD20阴性组。④CD20阳性组患者骨髓浆细胞比例（0.400对0.295，P=0.045）及恶性浆细胞比例（20.0%对6.8%，P=0.000）、CD45阳性细胞率（12.1%对4.5%，P=0.018）、CD79a阳性细胞率（9.8%对1.5%，P=0.013）、t（11;14）发生率（60.5%对14.4%，P=0.000）明显高于CD20阴性组。⑤两组患者对治疗的反应率、完全缓解率、无进展生存及总体生存时间差异均无统计学意义（P值均>0.05）。 结论 MM患者CD20阳性发生率为13.3%，CD20阳性MM患者的瘤细胞更易在骨髓浸润，累及红系造血，免疫表型更多为不典型表现。与CD20阴性患者比较，CD20阳性患者t（11;14）发生率明显升高，而t（4;14）发生率明显降低。OBJECTIVE To explore the clinical and laboratory characteristics of mutiple myeloma patients with CD20 expression. METHODS Review the data of mutiple myeloma patients and analyze the clinical and laboratory characteristics of CD20 positive patients, compared with CD20 negative patients. RESULTS (1)Totally 465 cases of newly-diagnosed MM were collected with CD20 expression status detected by multi-color flow cytometry. Sixty two patients (13.3%) were CD20 positive and the others were negative. (2)No statistical differences were found between CD20 positive and negative groups about the sex ratio, age predominance, D-S staging, ISS staging, renal insufficiency rate, platelet count, LDH level and classifications by paraprotein(all P value>0.05). (3)Compared with those of CD20 negative patients, the hemoglobulin value(74.5 g/L vs 83.5 g/L, P=0.021), extramedullary involvement rate (3.5% vs 13.7%, P=0.029), CD56-positive rate(36.7% vs 68.8%,P=0.000), t(4;14)translocation rate(2.4% vs 24.0%, P=0.001) in CD20 positive patients were lower statistically. (4)Compared with those of CD20 negative patients, the percentage of plasma cells (0.400 vs 0.295, P=0.045) by marrow smear differential counting, the percentage of myeloma cells(20.0% vs 6.8%, P=0.000) by multi-color flow cytometry analysis, CD45-positive rate(12.1% vs 4.5%, P=0.018), CD79a-positive rate(9.8% vs 1.5%, P=0.013) and t(11;14) translocation rate(60.5% vs 14.4%, P=0.000)in CD20 positive patients were higher statistically. (5)There was no statistical differences about the overall response rate (ORR), complete response rate (CRR), TTP(time to progression), PFS(progression free survival) and overall survival (OS) between CD20 positive and negative groups. CONCLUSION CD20 positive rate is 13.3% in multiple myeloma pateints according to our data. CD20 poaitive myeloma were prone to residing in bone marrow and affecting erythropoiesis. Atypical immunophenotypes were more common, and the incidence of t(11;14) were increased markedly while that of t(4;14)were rare for CD20 positive multiple myeloma.

[Outcomes of younger than 60 years old adults with Ph/BCR-ABL positive acute lymphoblastic leukemia: a single center clinical trial of BDH ALL 2000/02].

OBJECTIVE To explore the treatment options for younger than 60 years old adults with Ph /BCR-ABL positive acute lymphoblastic leukemia (Ph⁺ ALL). METHODS From January 2001 to June 2012, 42 adult patients were enrolled in the study. All patients received standard VDCP±L ±imatinb (IM) as induction therapy followed by intensive consolidation of modified Hyper-CVAD/MA±IM. At complete remission 1 (CR1), patients with appropriate donor received allogeneic hematopoietic stem cell transplantation (allo-HSCT), the others sequentially received intensive consolidation ±IM and autologous HSCT (ASCT) at molecular CR (MCR), then MM±VP±IM as maintenance therapy. Overall survival (OS), disease free survival (DFS) and relapse rate (RR) were analyzed. RESULTS CR rate after 1 cycle of induction chemotherapy was 83.3%. 39(92.9%) patients achieved CR. The median DFS and OS were (22.0±3.5) and (37.0±5.3) months respectively, with cumulative RR of (43.7±9.7)% during a median follow-up of 26.5(8-75) months. All 7 patients in CT group relapsed. Two patients received IM pre- and post-ASCT maintained MCR for 35 and 12 months after ASCT. But the other 3 ASCT recipients without IM died of relapse within 1 year. The transplant-related mortality rate in allo-HSCT group was 12.5%. The estimated 3-year OS in allo-HSCT (n=16), ASCT (n=5) and CT (n=7) groups were (66.7±12.2)%, (25.0±21.7)% and (16.7±15.2)%, respectively (P=0.014); meanwhile, the estimated 3-year DFS in those groups were of (56.3±12.4)%, (26.7±22.6)% and 0, respectively (P=0.002). CONCLUSION IM combined with intensive chemotherapy significantly increased the CR rate with the improved quality of CR, which highlighted the feasibility of SCT. Allo-HSCT could decrease relapse to produce favorable OS and DFS in CR1 of young adults with Ph⁺ ALL. ASCT combined IM might be the treatment of choice for those achieved MCR but without donors.

[Analysis of the efficacy and prognosis on first-line autologous hematopoietic stem cell transplantation of patients with multiple myeloma].

OBJECTIVE To explore the efficacy and prognosis of first-line autologous hematopoietic stem cell transplantation (ASCT) for newly diagnosed patients with multiple myeloma(MM). METHODS From January 2005 to December 31, 2012, 60 patients with MM were enrolled. All patients received thalidomide or/and bortezomib-based induction therapy, then received high-dose melphalan (200 mg/m²) and autologous stem cell support to get a ≥ partial response (PR), and followed by thalidomide-dexamethasone (TD) ±bortezomib as consolidation or maintenance treatment. With the follow up to December 31, 2012, the overall survival (OS), progression free survival (PFS) and the prognostic factors, including ISS stage, response and fluorescent in situ hybridization (FISH) data of cytogenetics were analyzed. RESULTS With a median follow up of 36.8 (12.0-102.5) months, the median OS and PFS estimate were not reached and 86.5 months, respectively. After transplantation, all (100%) patients received very good partial response (VGPR), and 34 (56.7%) patients achieved complete response (CR) after consolidation or maintenance treatment. The patients that achieved CR resulted in long term PFS (P=0.030), with no difference in OS (P=0.942). The univariate analysis showed that the abnormalities, including 13q14 deletion, 1q21 gain, IgH location and p53 deletion had the prognostic impacts. If the t(4;14) or p53 deletion was excluded, there would be no correlation between 13q14 deletion or 1q21 gain with PFS and OS. The patients with p53 deletion had a worst survival. CONCLUSION There has been significant improvement in the outcome for young MM patients by using ASCT and novel drugs. Cytogenetic abnormalities and response to therapy are the main factors affecting the survival of patients.

[Autologous hematopoietic stem cell transplantation in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia: a single center experience from the BDHALL2000/02 protocol].

目的 探讨自体造血干细胞移植（AHSCT）治疗年轻成人Ph−急性淋巴细胞白血病（ALL）的疗效。 方法 纳入56例于2000年1月至2007年12月接受BDHALL2000/02方案治疗并于CR1期行AHSCT的成人（15~60岁）Ph−ALL患者，对其进行生存和预后影响因素分析。 结果 56例患者中标危、中危和高危者分别为23例（41.1%）、19例（33.9%）和14例（25.0%）。中位随访75(7~177）个月。5年总生存（OS）、无事件生存（EFS）、无复发生存（RFS）、复发率分别为（51.8±6.7）%、（51.8± 6.7）%、（60.5±6.9）%、（39.1±6.9）%。标、中、高危组患者的5年OS率分别为（60.9±10.2）%、（52.6± 11.5）％和（35.7±12.8）%，EFS率分别为（60.9±10.2）%、（52.6±11.5）％和（35.7±12.8）%，RFS率分别为（68.3±9.9）%、（62.5±12.1）％和（44.9±14.1）%，复发率分别为（31.7±9.9）%、（37.5±12.1）％和（55.1± 14.1）%。标危和中危组、中危和高危组患者的上述指标比较差异均无统计学意义（P值均>0.05）；标危组患者的OS、EFS率高于高危组（P值分别为0.040和0.029），而RFS和复发率差异则无统计学意义（P值均>0.05）。对年龄≥35岁、完全缓解时间超过5周、初诊白细胞水平、免疫表型（B/T）、伴髓系表达、超二倍体染色体核型、复杂核型、完全缓解至AHSCT间隔时间、预处理方案是否包含TBI等进行单因素分析，均未显示对预后存在影响（P值均>0.05）。 结论 年轻成人Ph−ALL患者经BDHALL2000/02方案治疗可以获得较高的缓解率，缓解后给予早期序贯强化/巩固治疗后进行AHSCT疗效显著，是标危、中危组及无合适供者的高危组患者的合适选择。OBJECTIVE To evaluate the results of autologous hematopoietic stem cell transplantation (auto-HSCT) in adults with Philadelphia chromosome-negative acute lymphoblastic leukemia (Ph-ALL). METHODS From January 2000 to December 2007, the clinical data of auto-HSCT in adults Ph-ALL with complete remession (CR) 1 according to BDHALL2000/02 protocol were analyzed. RESULTS A total of 56 patients were enrolled and the probabilities of standard risk, intermediated risk and high-risk group were 41.1%, 33.9%, and 25.0%, respectively. After a median follow-up of 75 months (range 7-177 months), the 5-year overall survival (OS), events free survival (EFS) and relapse free survival (RFS) were (51.8 ± 6.7)%, (51.8 ± 6.7)%, and (60.5 ± 6.9)%, respectively. And the 5-year accumulative relapse rate was (39.1 ± 6.9)%. The 5-year OS of standard risk, intermediate risk, high-risk group were (60.9 ± 10.2)%, (52.6 ± 11.5)%, and (35.7 ± 2.8)%, respectively. The 5-year RFS among three groups were (68.3 ± 9.9)%, (62.5 ± 12.1)%, and (44.9 ± 14.1)%, respectively. The 5-year EFS among three groups were (60.9 ± 10.2)%, (52.6 ± 11.5)%, and (35.7 ± 12.8)%, respectively. The 5-year accumulative relapse rate among three groups were (31.7 ± 9.9)%, (37.5 ± 12.1)%, and (55.1 ± 14.1)%, respectively. There was no statistical significance of any survival rates between standard and intermediate risk groups, just as intermediate and high-risk groups. The OS and EFS in standard risk group were superior to those in high-risk group (P=0.040 and P=0.029, respectively), while there was no statistical significance of RFS and accumulative relapse rate between the two groups. The clinical factors listed below did not influenced the prognosis in the univariate analysis (P>0.05), including more than 5 weeks reaching to CR, WBC count at diagnosis, different immunophenotype (T or B cells), myeloid antigen expression, hyperdiploid chromosome karyotype, complex chromosome abnormality, conditioning regimen with or without TBI, duration between transplantation and diagnosis. CONCLUSION Ph-ALL adults could achieve a satisfactory CR and better survial according to BDHALL2000/02 protocol followed by auto-HSCT, especially for the standard or intermediate risk group, and no-donors high-risk patients.

[Clinical analysis of 25 patients with aggressive peripheral T-cell lymphoma in advanced stage treated with autologous stem cell transplantation].

目的 探讨大剂量化疗联合自体造血干细胞移植（ASCT）治疗侵袭性外周T细胞淋巴瘤（PTCL）的疗效。 方法 回顾性分析1997年5月至2013年6月于完全缓解（CR）期接受ASCT治疗的25例侵袭性PTCL患者的临床资料。 结果 ①25例患者中男16例，女9例，中位发病年龄30(12~54）岁。病理类型包括PTCL非特指型（PTCL-U) 16例、血管免疫母细胞淋巴瘤（AITL）4例、间变大细胞淋巴瘤（ALCL）3例和肝脾T细胞淋巴瘤（HSTL) 2例。临床分期Ⅲ期和Ⅳ期分别为8例和17例；其中合并骨髓侵犯者9例。移植前19例处于CR1期，另6例为CR2期。②2例HSTL均为骨髓侵犯、国际预后指数评分高危患者，联合化疗后获得CR1并行ASCT巩固治疗，但均于移植后12个月内早期复发、死亡。其余23例患者中位随访38(14~110）个月，3年无进展生存（PFS）率和总体生存（OS）率分别为（63.1±10.5）%和（71.8±9.9）%。CR1期（17例）移植者的生存有优于CR2期（6例）移植者的趋势[3年PFS率（74.9±11.0）％对（33.3±19.2）%，P=0.092; 3年OS率（80.2±10.4）％对（50.0±20.4）%，P=0.043]。无骨髓侵犯者的生存有优于骨髓侵犯者的趋势[3年PFS率（77.9±11.3）%对（40.0±17.4）%，P=0.142；OS率（84.4±10.2）％对（53.3±17.3）%，P=0.076]。 结论 ASCT改善化疗敏感侵袭性PTCL患者的生存。移植前非CR1状态和淋巴瘤骨髓侵犯可能是影响ASCT疗效的不良预后因素。HSTL患者预后差，ASCT后早期复发率高，需要进一步探索有效的治疗方案。OBJECTIVE To investigate the outcomes of autologous stem cell transplantation (ASCT) for patients with aggressive peripheral T-cell lymphoma (PTCLs) in advanced stage. METHODS The clinical data of 25 patients in complete remission (CR) with aggressive PTCLs received ASCT from May 1997 to June 2013 were retrospectively analyzed. RESULTS ① Of the 25 cases, 16 were unspecified PTCL (PTCL-U), 4 with angioimmunoblastic T cell lymphoma (AITL), 3 with anaplastic large cell lymphoma (ALCL) and 2 with hepatosplenic T cell lymphoma (HSTL), with a median age of 30(12-54) years old. Ratio of male to female is 16∶9. The distribution of stages was 8 cases with stage Ⅲ and 17 patients with stage Ⅳ. Nine patients presented with bone marrow involvement. Before ASCT, 18 patients were in CR1 and 7 patients were in CR2. ②Two patients with HSTL in stage ⅣB and IPI score 4/5 in CR1 relapsed and died within 12 months after ASCT. At a median follow-up of 38 (range 14-110) months, the estimated 3-year probability of PFS and OS for the other 23 patients was (63.1 ± 10.5)% and (71.8 ± 9.9)%, respectively. The patients in first CR had a better survival than the patients in second CR. The 3-year probability of PFS were (74.9 ± 11.0)% vs (33.3 ± 19.2)% (P=0.092) and OS were (80.2 ± 10.4)% vs (50.0 ± 20.4)% (P=0.043), respectively. The 3-year probability of PFS and OS were (40.0 ± 17.4)% and (53.3 ± 17.3)% in bone marrow involvement patients and the corresponding figure were (77.9 ± 11.3)% and (84.4 ± 10.2)% in non- bone marrow involvement patients. CONCLUSION ASCT could improve the survival of aggressive PTCLs. Non CR1 status and bone marrow involvement had negative influence on OS in patients with aggressive PTCLs treated by ASCT. The prognosis was very poor in patients with HSTL and satisfactory regimens should be investigated.

[Clinical and biological characteristics of non-IgM lymphoplasmacytic lymphoma].

目的 探索非IgM型淋巴浆细胞淋巴瘤（LPL）患者的临床及生物学特征，并与华氏巨球蛋白血症（WM）进行比较，探索两者的异同。 方法 对2000年1月至2013年12月收治的13例非IgM型LPL患者临床资料进行回顾性分析，应用荧光原位杂交技术（FISH）对其中7例患者标本进行检查。 结果 13例患者中，男7例，女6例，中位发病年龄63(43~74）岁。2例分泌单克隆IgA, 6例分泌单克隆IgG，5例不分泌单克隆性免疫球蛋白。以贫血为主要表现者7例，以皮肤黏膜出血和浅表淋巴结肿大为主要表现者各2例，出现B症状（发热、盗汗、体重减轻）者8例。所有患者均骨髓受累并表现贫血，10例患者为血常规2系或以上减少。行流式细胞术检查的5例患者中CD19、CD20、CD22和CD25均阳性，CD10、CD38和CD103均阴性，CD5弱阳性1例（该患者CD23阴性），sIgM阳性1例，CD23和CD11c阳性各2例，FMC7阳性3例。7例患者行细胞遗传学检查，未见异常核型，应用FISH检查发现其中2例患者伴有6q缺失。 结论 结合文献报道，非IgM型LPL与WM患者临床及生物学特征相似。OBJECTIVE To observe the clinical and biological characteristics of Non-IgM-secreting lymphoplasmacytic lymphoma (LPL) and draw the differences between non-IgM LPL and Waldenström macroglobulinemia (WM). METHODS Records of 13 patients with non-IgM LPL were retrospectively analyzed between January 2000 and December 2013. The cytogenetic aberrations were detected by fluorescence in situ hybridisation (FISH). RESULTS In the cohort, 7 males and 6 females with a median age of 63 years (range 43 to 74), two patients were IgA secreting, 6 with IgG secreting and 5 patients without monoclonal globulin. The major complaint at diagnosis included anemia associated symptom (53.8%), mucocutaneous hemorrhage and superficial lymphadenopathy (15.4%). Eight patients had B symptom at diagnosis. All of the 13 patients had bone marrow involvement and anemia, and 10 patients had 2 or 3 lineage cytopenia. In 5 patients with available immunophenotypic data, all expressed CD19, CD20, CD22 and CD25, but missed the expression of CD10, CD103 and CD38. Two cases had CD5 or sIgM positive alone. Another 2 patients were CD23 or CD11c positive and 3 patients were FMC7 positive. Cytogenetic aberrations had been detected by FISH in 7 patients, but only two (28.6%) patients had aberrations with del(6q). CONCLUSION The clinical and biological characteristics had no significantly difference between non-IgM LPL and WM.

[Clinical and laboratory features of T-cell prolymphocytic leukemia in China].

OBJECTIVE To investigate the clinical and laboratory characteristics and survival of Chinese patients with T- cell prolymphocytic leukemia (T-PLL). METHODS Eleven patients with T-PLL admitted in our hospital from Jan 2006 to Oct 2012 were retrospectively analyzed. RESULTS Of the 11 patients, nine were males and two females, with the median age of 56.0(19-69) years old. All the patients, except for three, presented with leukocytosis. The incidence of hyperleukocytosis (1/11) was less frequent than that in the British series (75%) (P=0.000). Lymphocyte counts in peripheral blood were increased in 9 of the 11 patients with the median absolute lymphocyte count (ALC) of 17.22(0.58-148.83)×10⁹/L. Superficial lymphadenopathy and splenomegaly were the most common physical signs. It was common that serum lactate dehydrogenase (LDH) and beta 2 microglobulin(β2-MG)were higher than normal level. All cases were positive for CD2/CD3/CD5/TCRαβ, negative for CD1a /HLA-DR and TdT, and most of them were strong positive for CD7 expression. By chromosome analyses, most cases. (9/10) have normal chromosome. This rate is significantly higher than that of the British and American series (3% and 25%, respectively) (P=0.000, P=0.001). The 14q11 abnormality and trisomy 8q, which are common among Western cases, were not observed in any of our cases. With a median follow-up of 23.0 months, three patients died. Two year progress free survival (PFS) and overall survival (OS) were 53.3% and 50%, respectively. There were 3 patients with PFS over a number of years, whether it should be considered as the T-chronic lymphocytic leukemia (T-CLL) is worthy of further studies. CONCLUSION The common clinical manifestations of T-PLL patients were increased lymphocyte counts and lymphadenopathy as well as splenomegaly. And most cases have high level of blood LDH and β2- MG and normal chromosome karyotype.

[Clinical efficacy and safety of chemoimmunotherapy with rituximab,fludarabine and cyclophosphamide for chronic lymphocytic leukemia].

OBJECTIVE To evaluate the efficacy and safety of a chemoimmunotherapy regimen of rituximab, fludarabine and cyclophosphamide (FCR) for patients with chronic lymphocytic leukemia(CLL). METHODS The clinical data of 26 CLL patients receiving FCR regimen in our hospital from April 2003 to January 2012 were analyzed retrospectively. Patients were grouped according to indicators including Rai risk stratification, β(2)-MG, LDH, ZAP-70, CD38, cytogenetics and immunoglobulin heavy chain variable region gene (IgVH) mutation status. Therapy efficacy and survival were evaluated and the safety of FCR regimen was assessed. RESULTS Among 26 patients, the overall response rate ( ORR ) was 76.9%, 10 patients (38.5%) achieved complete remission(CR) and 10(38.5%) partial remission(PR). With a median follow-up time of 30 ( 3-98 ) months, the median estimated progression-free survival(PFS) for all patients was 42(16-68) months and median overall survival(OS) was 63(41-85)months. Clinical parameters associated with higher CR rates were ＜2 courses of prior treatment regimens, proportions of bone marrow lymphocytes declining ≥ 50% after 2 courses of FCR, low LDH, low β(2)-MG and ZAP-70 negative (P = 0.014, 0.008, 0.027, 0.035 and 0.013, retrospectively). PFS and OS time in minimal residual disease(MRD)-negative, normal LDH and proportions of bone marrow lymphocytes declining ≥ 50% after 2 courses of FCR patients were significantly better than that of the control group (P＜0.05), PFS in the non-high-risk genetics group was significantly better than that in the high-risk genetics group (P = 0.005), while OS between two groups showed no statistically significant difference. The most common toxicities were gastrointestinal reactions (88.5%), followed by bone marrow suppression (80.8%): including neutropenia, anemia and thrombocytopenia. Infections accounted for 30.8%, mainly lung infection. CONCLUSION FCR is an effective and well-tolerated therapy for patients with CLL. Patients with MRD-positive, elevated LDH, proportions of bone marrow lymphocytes declining＜50% after 2 courses of FCR and high risk genetics patients are suitable for more effective treatment after achieving treatment response.