69例慢性淋巴细胞白血病的免疫表型及骨髓象分析

目的研究慢性淋巴白血病的免疫表型与骨髓象的特点,以期为临床诊治提供一定的参考。方法选取2013年1月至2015年1月间入院诊治的慢性淋巴细胞白血病患者69例,应用单参数和多参数流式细胞术分析患者的免疫表型,对患者进行骨髓象及血象检查,对患者的骨髓增生度进行分析。结果 69例CLL患者中,LL/SL-MC 33例(47.83%)、CLL/PLL-MC 16例(23.19%)、TSL 9例(13.04%)、ATLL 4例(5.80%)、PLL 4例(5.80%)、HCL 3例(4.34%)。CLL患者以年龄50岁以上者居多。骨髓增生度极度活跃12例(17.39%)、明显活跃10例(14.49%)、活跃47例(68.12%),粒红比增高17例(24.64%)、正常29例(42.03%)、降低23例(33.33%),巨核细胞计数增高3例(4.35%)、正常30例(43.48%)、降低36例(52.17%)。69例患者全部表达CD19、CD20,阳性率为100%;其次表达CD22,阳性率为98.55%;CD23阳性率为97.10%,CD5阳性率为86.96%;所有患者均不表达CD10。结论 69例CLL患者免疫分型以混合型为多,骨髓增生度较高;免疫表型则以CD19、CD20全部表达,其次分别表达为CD22、CD23与CD5,不表达CD10。     

Ecto-5'-nucleotidase in B-cell chronic lymphocytic leukemia.

Literature on the behaviour of ecto-5'-nucleotidase in the course of B-cell chronic lymphocytic leukemia is briefly reviewed and aims for further researches are highlighted.     

A 57-gene expression signature in B-cell chronic lymphocytic leukemia

B-CLL is the most frequent type of leukemia in the Western countries. The disease, common among the elderly, follows a variable course in terms of survival time and symptoms. There is evidence that the accumulation of lymphocytes in peripheral blood and bone marrow is due to a cell resistance to apoptosis rather than to highly proliferative cells. Genetic mechanisms that lead to the development and progression of disease are mainly unknown, although a number of prognostically and diagnostically important genetic markers have been identified.     

Effectiveness of allogeneic bone marrow transplantation in patients with acute leukemia in complete remission and in patients with chronic myeloid leukemia during the chronic phase]

AIM: To study clinical efficiency of allogenic bone marrow transplantation (ABMT) in patients with acute leukemia (AL) in the first remission and in patients with chronic myeloid leukemia (CML) in chronic phase; to analyse overall and recurrence-free survival in relation to the diagnosis and age. MATERIALS AND METHODS: 26 patients with AL and 24 ones with CML (29 males and 21 females) were subjected to ABMT within 10 last years. Median of age in AL and CML was 24.5 and 25.5 years, median of the time since the diagnosis was 9 and 13 months, respectively. Follow-up since the ABMT made up 67.5 months (31-107) and 38 months (6-108), respectively. Conditioning was made with cyclophosphamide (120 mg/kg) plus total radiation of the body (12 Gy) in 16 patients, myelosan (mileran) in a dose 16 mg/kg plus cyclophosphane (120 mg/kg) in 34 patients. The marrow was taken from HLA-identical sibs, enzygotic twins (5 recipients). Cytogenetic investigations were made in CML. The retention of the transplant was controlled by immunological and molecular tests. RESULTS: Among AL patients 50% are still alive. Probability of 80-month survival reached 55%, 110 months--42%. Probable recurrence-free survival was 78%. All the patients are in a complete clinico-hematological remission. Among CML patients 75% are still alive. Of them 89% had a complete hematological remission, 72% are in a complete hematological and cytogenetic remission. Probable 110 month survival equals 75%, probability to survive without recurrence--52%. Early lethality (100 days) of toxic and infectious complications was as low as 10 and 6%, respectively. Frequency of lethal acute secondary disease was under 8%. CONCLUSION: ABMT made in AL patients during the first complete remission and in CML patients in the chronic phase brings about very good results which are much better than after routine cytostatic chemotherapy.     

Association of health-related quality of life with gender in patients with B-cell chronic lymphocytic leukemia

Purpose

This analysis examined associations between gender and health-related quality of life (HRQOL) in patients with B-cell chronic lymphocytic leukemia (CLL) as they initiate therapy for CLL outside the clinical trial setting.

Methods

Baseline data were collected as part of Connect® CLL Registry, a prospective observational study initiated in community, academic, and government centers. Patient demographics and clinical characteristics were provided by clinicians. Patients reported HRQOL using the Brief Fatigue Inventory (BFI), EQ-5D, and Functional Assessment of Cancer Therapy-Leukemia (FACT-Leu). Mean scores were analyzed, with statistical significance of differences determined by ANOVA. Multivariate analysis also considered age and line of therapy.

Results

Baseline HRQOL data were available for 1,140 patients: 710 (62 %) men and 430 (38 %) women from 161 centers. Patients were predominantly white (89 %) with mean age 69?±?11 years. Women reported significantly worse global fatigue (P <0.0001), fatigue severity (P <0.0001), and fatigue-related interference (P?=?0.0005) versus men (BFI). Pain/discomfort (P?=?0.0077), usual activities (P?=?0.0015), and anxiety/depression (P?=?0.0117) were significantly worse in women than in men (EQ-5D). With women reporting a better social/family score (P?=?0.0238) and men reporting a better physical score (P?=?0.0002), the mean FACT-G total score did not differ by gender. However, the mean FACT-Leu total score was better among men versus women (P?=?0.0223), primarily because the mean leukemia subscale score was significantly better among men (P <0.0001). Multivariate analysis qualitatively confirmed these findings.

Conclusions

Connect® CLL Registry results indicate that significant differences exist in certain HRQOL domains, as women reported greater levels of fatigue and worse functioning in physical domains.     

Spontaneous remission of large granular lymphocytic leukaemia

The phenomenon of spontaneous remission is one of the less well known aspects of the leukaemias. Spontaneous remission has been reported in both acute and chronic leukaemia. The mechanism is unclear but may involve immune modulation related to an intercurrent infection or transfusion of blood products. We present a rare case of spontaneous complete remission of T-cell large granular lymphocytic (T-LGL) leukaemia occurring in an 82-year-old man. The patient died two years later of an unrelated condition, with no evidence of recurrence of T-LGL leukaemia. Elucidation of the mechanism of the phenomenon of spontaneous remission may lead to advances in the treatment of this diverse group of disorders.     

分化抗原簇CD38在慢性淋巴细胞白血病T淋巴细胞中的表达研究

目的 探讨慢性淋巴细胞白血病(CLL)中T淋巴细胞CD38(CD38-T)的表达特征.方法 应用多参数流式细胞术检测83例CLL患者肿瘤细胞中CD38表达(CD38-B)、zeta链相关蛋白-70(ZAP-70)以及CD38-T、CD4/CD8比值的表达情况.结果 在所有患者中,CD38+-T占49.4%(41/83),CD38+-B占50.6%(42/83),CD38+-T和CD38-B表达有高度相关性(r=0.553,P<0.01);表达ZAP-70+CD38+-T和ZAP-70-CD38+-T的患者占67.5%(56/83),CD38-T与ZAP-70的表达有相关性(r=0.349,P<0.01).Binet A期患者中CD38+-T占33.3%(14/42),Binet B+C期患者中CD38+T占65.9%(27/41);XD38+-T与临床分期,CD4/CD8比例倒置有相关性(r=0.312、0.453,P<0.05).结论 CD38-T表达变化与患者病情进展及转归密切相关,CD38高表达的患者多伴有免疫功能调节紊乱或失衡,可作为CLL的一项全新独立的监测指标.     

Effect of interferon-alpha on CD20 antigen expression of B-cell chronic lymphocytic leukemia

Chimeric CD20 monoclonal antibody as alternative therapy in relapsed low-grade non-Hodgkin's lymphoma (NHL) has produced responses in nearly 50% of patients. Augmenting CD20 expression on tumor cells and/or inducing its expression may increase the cell kill and effectiveness of antibody therapy. Peripheral blood lymphocytes from 19 patients with B-cell chronic lymphocytic leukemia (B-CLL) were incubated in vitro in the presence of interferon-alpha (IFN-alpha) (500 U/ml and 1,000 U/ml) for 24 and 72 hours. The effect on CD20 expression was studied by flow cytometry. The differences in the percentage positivity, the mean fluorescence intensity (MFI), and the product of percentage positivity and MFI were used to assess upregulation. There was a significant upregulation of CD20 expression on B cells seen at both concentrations after 24-hour priming (p < 0.01). B-CLL cells cultured for 72 hours in the presence of IFN-alpha also showed upregulation of CD20 expression; however, the degree of upregulation was much lower than that seen at 24 hours. There was no statistically significant increase in CD20 antigen expression on normal lymphocytes following cytokine exposure. These results suggest that IFN-alpha priming may augment the effectiveness of antibody therapy by directly upregulating CD20 antigen expression in addition to its indirect action through effector cells of the host.     

Comparison of bone marrow and peripheral blood ZAP-70 status examined by flow cytometric immunophenotyping in patients with chronic lymphocytic leukemia

BACKGROUND:: The mutational status of the immunoglobulin heavy chain variable gene in patients with chronic lymphocytic leukemia correlates with prognosis. Patients with mutated IgVH genes fare better than those with unmutated genes. Gene expression profiling studies identified the tyrosine kinase ZAP-70 to be expressed in unmutated CLL samples. Flow cytometric examination of ZAP-70 expression in tumor cells has been proposed to be a convenient surrogate marker for IgVH mutational status. However, a few studies have shown a small number of discordant results between ZAP-70 positivity, IgVH mutational status, and clinical outcome. There have been no reported studies comparing bone marrow samples with peripheral blood for ZAP-70 expression in CLL patients. METHODS:: We searched our flow cytometry files from October 2004 through April 2006 and identified CLL in 311 bone marrow and peripheral blood specimens from 256 patients. We defined ZAP-70 positivity as greater than 30% of the CD19(+) B-cells above the isotype control value that coexpress ZAP-70. Statistical analyses were performed using the Fisher exact test and student t-test. RESULTS:: A significantly greater number of bone marrow specimens were positive for ZAP-70 when compared with the number of peripheral blood specimens. Of all the ZAP-70 negative specimens, CLL cells from bone marrow had a greater mean percentage of ZAP-70 positive cells when compared with the CLL cells from peripheral blood. Finally, six patients were identified who were ZAP-70 positive in the bone marrow but ZAP-70 negative in the peripheral blood. CONCLUSIONS:: These results may be due to either an increase in the false positive rate in bone marrow specimens or to an intrinsic feature of CLL cells in the compartment that is biologically distinct from peripheral tumor cells. As prognosis and treatment decisions may be based on ZAP-70 results from either specimen type, it is prudent to further examine this observation. (c) 2006 International Society for Analytical Cytology.