首页 | 官方网站   微博 | 高级检索  
相似文献
 共查询到20条相似文献,搜索用时 0 毫秒
1.
BackgroundFamilial clustering of B-cell disorders among Waldenström's macroglobulinemia (WM) patients has been reported, though the frequency and any differences in disease manifestation for familial patients remain to be defined.Patients and methodsWe therefore analyzed clinicopathological data from 257 consecutive and unrelated WM patients. Forty-eight (18.7%) patients had at least one first-degree relative with either WM (n = 13, 5.1%), or another B-cell disorder including non-Hodgkin's lymphoma (n = 9, 3.5%), myeloma (n = 8, 3.1%), chronic lymphocytic leukemia (n = 7, 2.7%), monoclonal gammopathy of unknown significance (n = 5, 1.9%), acute lymphocytic leukemia (n = 3, 1.2%) and Hodgkin's disease (n = 3, 1.2%). Patients with a familial history of WM or a plasma cell disorder (PCD) were diagnosed at a younger age and with greater bone marrow involvement.ResultsDeletions in 6q represented the only recurrent structural chromosomal abnormality and were found in 13% of patients, all non-familial cases. Interphase FISH analysis demonstrated deletions in 6q21-22.1 in nearly half of patients, irrespective of familial background.ConclusionsThe above results suggest a high degree of clustering for B-cell disorders among first-degree relatives of patients with WM, along with distinct clinical features at presentation based on familial disease cluster patterns. Genomic studies to delineate genetic predisposition to WM are underway.  相似文献   

2.
目的:研究抗CD20单克隆抗体利妥昔(rituximab)治疗华氏巨球蛋白血症(Waldenstrom macroglobu-linemia,WM)时血清IgM异常增高现象及其表现和处理,并复习相关文献,推荐处理方法。方法:1例68岁患者通过骨髓细胞学、病理学和免疫固定电泳等检查确诊为WM,并给予利妥昔(375 mg/m2,d1)联合复达拉滨(30mg/m2,i.v.d1-3)作为初始治疗,对治疗前后的血清IgM水平进行监测。结果:在第一疗程RF治疗结束后四周,患者的血清IgM水平从治疗前的34.8g/L增高至115g/L,同时出现头痛和眼底出血,但骨髓浆细胞和淋巴样浆细胞无明显增多。在停用利妥昔,改用FC(复达拉滨和环磷酰胺)方案治疗四疗程后,IgM下降至治疗前水平,然后进一步下降达部分缓解。结论:WM患者在接受以利妥昔为基础的治疗后,血清IgM可明显增高,并导致高黏滞综合征等高IgM相关并发症。尽管此种异常增高并不提示利妥昔单抗治疗失败,但在治疗过程中仍需密切。在治疗开始的前二个疗程中避免使用利妥昔单抗可减少此现象的发生。  相似文献   

3.
BACKGROUND: The anti-CD20 monoclonal antibody rituximab is an important therapeutic in Waldenstrom's macroglobulinemia (WM), producing response rates of 50-70%. Responses, which are based on serum IgM levels, have typically been evaluated at 12 weeks. Paradoxically, we have observed that serum IgM levels can abruptly rise following rituximab therapy in patients with WM, and can often lead to morbidity on the basis of hyperviscosity. PATIENTS AND METHODS: Eleven WM patients with CD20+ tumor cells who received rituximab at our Institution and had serum IgM levels measured within a 12-week period following start of therapy were evaluated. Therapy consisted of four weekly infusions of rituximab at 375 mg/m(2). Pre- and post-therapy serum IgM levels were determined by nephelometry and corresponding serum viscosity levels were determined by viscometry. RESULTS: Ten of the 11 patients demonstrated an abrupt rise in serum IgM levels, with a >25% increase occurring in eight (73%) patients. Mean serum IgM levels for all 10 spiking patients rose from 4370 (range, 655-7940) to a peak of 5865 (range, 872-11 800) mg/dl (P=0.004), which occurred at a mean of 4 (range, 1-8) weeks following initiation of therapy. Mean serum viscosity levels also increased from 3.5 to 5.6 centipoise (CP) (P=0.09) in eight patients for whom pre- and post-therapy studies were obtained. A subdural hemorrhage occurred in one patient when serum IgM levels rose from 7530 to 11 800 mg/dl, and serum viscosity increased from 3.9 to 10.1 CP. Two other spiking patients with pre-therapy IgM levels of >5000 mg/dl experienced worsening headaches and/or epistaxis attributed to increasing serum viscosity. CONCLUSIONS: Abrupt increases in serum IgM levels commonly occur following rituximab therapy in WM. Careful clinical and laboratory monitoring is warranted, particularly if patients have pre-therapy serum IgM levels of >5000 mg/dl. The mechanism of this effect is under active investigation, and may be related to CD20 signaling triggered by rituximab.  相似文献   

4.
BACKGROUND: To quantify the long-term risk of second cancers (SCs) up to 30 years after primary treatment for Hodgkin's disease (HD) Material and methods In the period 1968 to 1985, an unselected population of 1024 patients started treatment for HD at the Norwegian Radium Hospital (NRH) and were followed for SC from 1969 through 1998 by The Norwegian Cancer Registry. The median age at diagnosis of HD was 40 years, and the median time at follow-up was 14 years. RESULTS: Of 197 SCs, 14 were acute non-lymphocytic leukemia (ANLL), 31 non-Hodgkin's lymphoma (NHL) and 152 solid cancers. The standardized incidence ratio (SIR) was significantly increased for SCs as a group, and for the subgroups ANLL, NHL, lung cancer, breast cancer, stomach cancer and melanoma. ANLL was related to heavy treatment with chemotherapy (CT) and combined CT and radiotherapy (RT), NHL was not treatment related, and solid tumors were related to radiotherapy only or combined RT and CT. The SIR of ANLL and NHL reached a peak between 5 and 10 years after treatment. Solid and non-solid tumors increased with young age at diagnosis of HD and solid tumors increased with follow-up time up to 28 years CONCLUSION: In a long-term follow-up study of HD patients of all ages, the SIR of solid tumors was high in patients treated at young age and decreased with increasing age. Most solid tumors had started within or at the edge of the irradiated field, and SIR of solid tumors increased even 20-30 years after diagnosis.  相似文献   

5.
BACKGROUND: The aim of this study was to explore variation in practice patterns and identify factors associated with physicians' treatment decisions for early-stage Hodgkin's disease. METHODS: We conducted a one-time mail survey of oncologists randomly selected from directories of national oncology societies (n = 207) and Hodgkin's disease experts (n = 147). The survey included questions on (i) physician factors, (ii) preferred treatment choices for six case scenarios of early-stage Hodgkin's disease that varied by patient factors, and (iii) thresholds for changing treatment recommendations. RESULTS: The response rate was 50%. For non-bulky Hodgkin's disease, 69% of respondents chose combined modality therapy (CMT). On multivariate analysis, physician factors that independently predicted for choice of CMT included a high Hodgkin's disease case load (P = 0.02) and a high percentage of patients enrolled in clinical trials (P = 0.05). Radiation oncologists had a lower threshold for adding radiation therapy (P = 0.02). More experience with second malignancy cases and longer time in practice were associated with a higher threshold for adding radiation therapy (P = 0.04 and P = 0.008, respectively). In stratified analyses, treatment decisions of non-experts were significantly influenced by physician factors, but not by patient factors. Conversely, choices of Hodgkin's disease experts were insensitive to all physician factors, but experts were significantly more likely to select chemotherapy alone in young women and CMT in older patients. CONCLUSIONS: Our results indicate that physician factors including practice type and experience may in part explain variation in practice pattern for Hodgkin's disease therapy. Hodgkin's disease experts are more likely to tailor therapy according to individual patient factors.  相似文献   

6.
BACKGROUND: Many papers have reported the results achieved with combined therapy for Ewing's tumors, but little is known about the treatment and outcome of those 30-40% of patients who relapse. PATIENTS AND METHODS: In a retrospective study, we evaluated 195 patients with Ewing's tumors treated at our institution from 1979 to 1997 with chemotherapy, radiotherapy, surgery or combined therapies after recurrence. RESULTS: A second complete remission was achieved in only 26 patients (13.3%); 12 relapsed again and died of the tumor. The 5-year post-relapse event-free survival and overall survival were 9.7% and 13.8%, respectively; both of which were significantly better for patients who had relapsed >/=2 years after the beginning of the first treatment (14.3% versus 2.5%; P <0.001) and for patients who relapsed with only lung metastases (14.5% versus 0.9%; P <0.0005). In terms of treatment, patients treated with surgery or radiotherapy, alone or in combination with chemotherapy, had better survival rates than patients treated with chemotherapy alone (15.4% versus 0.9%; P <0.0001). CONCLUSIONS: The outcome of Ewing's tumor patients who relapse after combined treatment is very poor. However, these patients may be divided into two groups: those that can be cured with traditional treatments (late relapse and/or only lung metastases), and a second group of patients (early relapses with metastases in lungs and/or other sites) who gain no benefit from traditional therapies. For the latter group, multicenter studies are needed to evaluate new strategies of treatment.  相似文献   

7.
Waldenstrom's macroglobulinemia is an indolent B-cell malignancy that is characterized by high levels of IgM paraprotein production and is incurable with standard chemotherapy. Iodine 131I-Tositumomab (iodine-131-labeled murine anti-CD20 monoclonal antibody; Bexxar) is a novel radioimmunotherapeutic agent that has a high response rate in relapsed or chemotherapy refractory, CD20-positive, low grade or transformed B-cell non-Hodgkin's lymphomas. There are no data on the use of radioimmunotherapy in Waldenstrom's macroglobulinemia. We report a patient with Waldenstrom's macroglobulinemia with transformation to a large B-cell lymphoma, who was treated successfully with iodine 131I-tositumomab. The patient had a complete response to the treatment, including disappearance of any detectable IgM paraprotein. This case report demonstrates the potential for radioimmunotherapy in CD20 positive B-cell malignancies.  相似文献   

8.
BACKGROUND: Recent Wilms' tumor (WT) trials and studies have tried to determine the minimal therapy needed for cure. The goal was survival without morbidity. PATIENTS AND METHODS: From January 1989 to March 1994 the German Society of Pediatric Oncology and Hematology registered 440 patients (median age 2.9 years; 231 male, 209 female) with WTs (preoperative chemotherapy 362) for therapy according to the International Society of Pediatric Oncology Trial and Study 9. Therapy for relapse depended on site of relapse and therapy already received. Follow-up included inquiries for morbidity. Prognostic factors for relapse and death were evaluated. RESULTS: Five-year survival of WTs was 89.5%; 98.2% (385 of 392) of survivors had a follow-up of 5 years (range 0.8-12.6; median 8). In non-anaplastic WTs, young age (<2 years) was of significance (P = 0.026) for a better survival. Non-anaplastic WTs (407 patients) had a 5-year survival of 92.3%, versus 48.5% in anaplastic WTs (33 patients), and a 5-year relapse-free survival of 87.6% versus 42.4%. Survival after relapse was significantly worse for anaplastic than for non-anaplastic WTs (residual 3-year survival 11.8% versus 54.3%; P <0.0001). In preoperatively treated WTs, anaplasia was a strong prognostic factor for death [relative risk (RR) 4.7], followed by poor response to preoperative therapy (RR 3.6), stage IV (RR 3.2) and abdominal stage III (RR 2.2). Low abdominal stages (相似文献   

9.
BACKGROUND: In advanced age the prognosis of Hodgkin's lymphoma (HL) is poor, but, as a consequence of the low incidence of HL in the elderly, prospective studies are lacking and the best treatment strategy is difficult to define. PATIENTS AND METHODS: One-hundred and five HL patients over 65 years of age were treated homogeneously with an original reduced-intensity regimen designed for HL in the elderly containing vinblastine, cyclophosphamide, procarbazine, etoposide, mitoxantrone and bleomycin (VEPEMB). Forty-eight early stage (IA-IIA) patients received three courses of VEPEMB followed by involved field irradiation. Fifty-seven advanced stage (IIB-IV) patients received six courses followed by radiotherapy limited to the areas of bulky disease. RESULTS: Mean age was 71 years (range 66-83). Co-morbidities were present in 39 patients (37%). A treatment plan modification for poor tolerance or toxicity was needed in 18 patients. Results were satisfactory, even if they were better in early rather than in advanced stage disease: complete response rate 98% versus 58% (P <0.01); 5-year failure-free survival 79% versus 34% (P <0.01). The results were affected by advanced stage, systemic symptoms and co-morbidity but they were not influenced by age itself. CONCLUSIONS: VEPEMB is an effective and low toxic regimen for HL in the elderly. Co-morbidity is a prognostic factor more important than age itself.  相似文献   

10.
To assess the preliminary efficacy of rituximab therapy in Waldenstrom's macroglobulinemia (WM), we examined the clinical and laboratory data for all patients with WM treated on IDEC Pharmaceuticals sponsored trials and one patient treated at Walter Reed Army Medical Center. Seven symptomatic patients with WM were treated with four (n = 6) or eight (n = 1) weekly infusions of rituximab (375 mg/m2). Patients had received a median of three prior therapies (range 1–4) which included alkylator therapy in all (five patients refractory) and fludarabine in four (all refractory). Therapy was tolerated well in all patients without decrement in cellular immune function or significant infectious morbidity. Partial responses were noted in three of these patients, including two with fludarabine-refractory disease. The median progression-free survival for these patients was 6.6 months (range 2.2–29+ months). These data suggest that rituximab has clinical activity in heavily pre-treated patients with Waldenstrom's macroglobulinemia. Based on these data, clinical studies of Rituximab in previously untreated and treated WM appear indicated.  相似文献   

11.
Recent data have suggested that rituximab is an active agent for the treatment of Waldenstrom's macroglobulinemia (WM). However, the patients that are more likely to benefit have not been clearly defined. In order to address this question we evaluated 52 patients who were treated with single-agent rituximab in the context of prospective studies. Several clinical and laboratory variables were assessed for their correlation with response and time to progression. Twenty-three (44%) patients achieved a partial response after treatment with rituximab. Previously untreated and pretreated patients had the same probability for response. Higher response rates were noted in patients with serum monoclonal protein < 40 g/l, with serum albumin > or = 35 g/l and with kappa light chain. The median time to progression for all patients was 13.8 months. A multivariate analysis indicated that elevated serum monoclonal protein levels and low serum albumin were the dominant variables associated with shorter progression. Presence of two, one or none of these adverse prognostic factors was associated with time to progression of 3.6 months, 11 months and more than 40 months, respectively. We conclude that rituximab is an effective treatment modality for patients with WM. Patients with both low levels of monoclonal protein and normal albumin are the best candidates for treatment with standard dose rituximab.  相似文献   

12.
Postpneumonectomy syndrome (PPS) is a rare complication of pneumonectomy due to an excessive mediastinal shift producing compression of the main bronchus or a lobe bronchus on the aorta or the spine. We report an exceptional case in which an extreme mediastinal shift was due to fibrosis and complete atelectasis of the left lung, as a complication of chemoradiation treatment for recurrent mediastinal Hodgkin's lymphoma. This condition, associated with a further recurrence of the disease, indicated a postpneumonectomy-like syndrome.  相似文献   

13.
目的:探讨三阴性乳腺癌(triple-negative breast cancer,TNBC)的临床特点和预后情况。方法收集癌症和肿瘤基因图谱(The Cancer Genome Atlas,TCGA)数据库700例乳腺癌患者资料。根据分子标志物和激素受体状态,将其分为多种亚型。对三阴性和非三阴性乳腺癌临床数据进行Kaplan-Meier生存分析及χ2检验,预后影响因素采用单因素和多因素分析。结果生存曲线显示,TNBC 患者(n=85)与非三阴性乳腺癌(non triple-negative breast cancer,nonTNBC)患者(n =615)的10年总生存率(overall survival,OS )比较差异具有统计学意义(P=0.005),并低于luminal A型乳腺癌患者(P=0.001),与其他亚型患者比较差异均无统计学意义(均P>0.05)。TNBC与nonTNBC患者在绝经状态、组织类型、AJCC肿瘤病理状态、AJCC淋巴结病理状态和年龄方面比较差异均具有统计学意义(均P<0.05)。综合单因素及多因素分析发现,淋巴结转移是TNBC重要的独立预后因素(P=0.021)。伴随肿瘤患者生存时间也短于无肿瘤患者(P=0.009)。结论 TNBC临床病理特征与其他类型的乳腺癌差异较大。手术完全清除肿瘤对TNBC患者意义重大。  相似文献   

14.
BACKGROUND: The International Prognostic Score (IPS) and circulating levels of the soluble form of CD30 molecule (sCD30) have both been associated with poor outcome in patients with advanced Hodgkin's lymphoma (HL). The aim of this study was to assess the prognostic power of the combined evaluation of sCD30 and IPS in these patients. PATIENTS AND METHODS: We included 101 patients with advanced HL, treated with ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine) or MOPP (mechlorethamine, vincristine, procarbazine and prednisone)/ABVD chemotherapy with or without radiotherapy. All were tested for pre-treatment sCD30 levels. RESULTS: Six-year estimated overall survival (OS) and failure-free survival (FFS) was 89% +/- 3% and 75% +/- 4%, respectively. Thirty-three patients (33%) had IPS >2; their FFS was 60% compared with 82% in the remaining patients (P = 0.027). Serum sCD30 levels were > or =100 U/ml in 41 (41%) patients; their FFS at 6 years was 58%, compared with 87% in patients with sCD30 <100 U/ml (P = 0.003). In the 18 patients with both sCD30 > or =100 U/ml and IPS >2, FFS was significantly worse (44%) than in patients with low sCD30 and low IPS (89%) (P <0.001) or with only one of the two adverse prognostic factors (73%) (P = 0.03). CONCLUSIONS: In our study, the combination of IPS >2 and serum sCD30 levels > or =100 U/ml identifies a sizeable subgroup (18%) of advanced HL patients with very poor FFS, who might take advantage of intensified up-front treatment strategies.  相似文献   

15.
BACKGROUND: Conflicting data on the effect of the Epstein-Barr virus (EBV) on outcome in Hodgkin's disease (HD) might be due to the heterogeneous nature of this disease. In this study we have investigated whether the effect of EBV status on outcome is different between aetiologically defined age groups (15-34, 35-44, 45+ years) and also between males and females. PATIENTS AND METHODS: Paraffin-embedded sections from 273 patients with advanced HD from two related clinical trials were analysed for the presence of EBV using in situ hybridisation. RESULTS: EBV was detected in 78 (29%) of cases. For all patients, after a median follow-up of 5 years, there were no significant differences in survival by EBV status although there was a trend towards longer failure-free survival times for EBV-positive patients. Multivariate analyses suggested that EBV and sex, when in combination, were prognostic factors for failure-free survival (P = 0.06 for both). For subgroups, the effect of EBV on failure-free survival was significant for males and 15-34 years age group (P = 0.05 and P = 0.03, respectively). CONCLUSION: This study suggests that with a median follow-up of 5 years, EBV status does not affect survival but being EBV-positive may be beneficial in terms of failure-free survival, particularly for males and younger adults.  相似文献   

16.
 目的 探讨原发性巨球蛋白血症(Waldenstrom’s macroglobulinemia,WM)的临床特点、免疫表型、遗传学改变及诊疗措施。方法 回顾分析一例原发性巨球蛋白血症患者的临床资料。结果 本病例经血清固定电泳、骨髓形态学、细胞免疫分型等检测结果证实为原发性巨球蛋白血症,患者经过8个疗程的化疗后,恢复良好,目前仍在定期随访之中。结论 WM有特殊的免疫表型及细胞遗传学的改变,在临床工作中如果遇到单克隆IgM增高的病例,建议要考虑到WM的可能性,进一步完善免疫表型和细胞遗传学检测。  相似文献   

17.
18.
BACKGROUND: Elderly patients with acute myeloid leukemia (AML) generally have an unfavorable clinical course and are under-represented in clinical trials. The aim of this study was to analyze the prognosis and treatment outcome of elderly AML patients. PATIENTS AND METHODS: We studied 205 AML patients aged 65 years or older at our hospital. Prior to study initiation, we designated 13 variables to be analyzed for their impact on complete remission (CR) rate and overall survival (OS). RESULTS: Induction regimen (standard chemotherapy) and good performance status (PS) (Eastern Cooperative Oncology Group PS 0-1) independently influenced the achievement of CR. Multivariate analysis also determined five poor prognostic factors for OS: poor PS (score 2-4), presence of comorbidities, elevated serum lactate dehydrogenase level (> or =2x upper normal limit), extreme leukocytosis (> or =100 x 10(9)/l) and marked thrombocytopenia (< or =20 x 10(9)/l). Age was not an independent contributing factor in terms of either CR attainment or OS duration. Low-risk patients, who possessed one or less non-leukocytosis poor prognostic factor, had significantly longer disease-free survival and OS than their high-risk counterparts. CONCLUSIONS: Elderly AML patients should be risk-stratified at diagnosis. Anthracycline-based induction chemotherapy would be the best therapeutic option for such patients.  相似文献   

19.
BACKGROUND: The most commonly used regimen for the treatment of advanced Hodgkin's disease (HD) is ABVD (doxorubicin, bleomycin, vinblastine and dacarbazine). Two of these components, bleomycin and dacarbazine, have defined toxicities such as pulmonary fibrosis and nausea/vomiting, and also uncertain single-drug activity. The EVA regimen (etoposide, vinblastine, doxorubicin) is an attempt to substitute a known active agent, etoposide, for bleomycin and dacarbazine. PATIENTS AND METHODS: A series of 51 patients with advanced HD without prior systemic therapy were treated. The series included 12 stage II patients with bulky (>10 cm) mediastinal tumors, 10 of whom received complementary radiation therapy. The remaining patients received EVA only. Response, duration of response, survival, toxicity and the efficacy of salvage therapy were evaluated in all patients. The median follow-up time was 111 months and permitted an assessment of the long-term effects of treatment and natural history of a cohort of treated patients. RESULTS: EVA achieved a complete response (or clinical complete response) in 48/51 patients (94%). Of these 48 responders, 16 relapsed in a median of 11 months (range 3-48 months). In follow-up, 32/51 patients had no evidence of relapsed HD, although three died from other causes (two from vascular events and one from large cell lymphoma), resulting in progression-free survival for the entire group of 57% at 111 months. Eight of the 16 were alive and free from disease at follow-up at 111 months. In the entire series, only seven patients (14%) died of HD. 37 patients (73%) continued free from disease. There was no pulmonary toxicity. CONCLUSIONS: The EVA regimen appears to have an overall survival (OS) outcome comparable to ABVD, but without the lung toxicity. The high salvage rate of second-line therapy, in most instances at conventional dosage, suggests an absence of cross-resistance to alkylating agents in patients treated with EVA.  相似文献   

20.
目的:探讨晚期结直肠癌姑息化疗后获得疾病稳定患者的预后异质性及个体化治疗策略的应用。方法:研究收集2008年4 月至2014年10月204 例在天津医科大学肿瘤医院接受标准一线和(或)二线化疗后获得疾病稳定的晚期结直肠癌患者,分析该人群临床病理特征,筛选预后异质性分层因子,并评估姑息化疗后治疗手段(即单纯观察或维持治疗)对于特殊人群的影响。结果:单因素生存分析表明化疗线数、基线CA724、CEA 、CA19- 9 水平、血小板淋巴细胞比率(PLR )、姑息化疗后治疗手段为预后影响因素。多因素Cox 比例风险回归模型分析显示,一线化疗、基线CA19- 9 低水平、PLR 低水平、姑息化疗后维持治疗患者预后较好。亚组分析显示PLR 高水平患者中,维持治疗组与观察组无进展生存期分别为13.43个月和10.63个月(P = 0.003)。 结论:基线CA19- 9、PLR 水平及姑息化疗后治疗手段为获得疾病稳定的晚期结直肠癌患者的独立预后因子。疾病稳定人群尤其是PLR 高水平者宜接受维持治疗。  相似文献   

设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司    京ICP备09084417号-23

京公网安备 11010802026262号