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1.
Thirty-one patients with newly diagnosed acute lymphoblastic leukemia were examined before receiving any treatment and their clinical and laboratory data were analyzed in order to determine the possible correlation between clinical presentation, morphologic sub-classes, cytochemical reactions, immunological phenotypes and cytogenetic findings. Each of the previous parameters and response to therapy were also examined for correlation. The analysis of clinical and laboratory characteristics of patients according to their immunological phenotype did not show any significant male sex bias, age distribution, hepatomegaly or splenomegaly at diagnosis. The analysis of clinical response of patients did not demonstrate any significant influence of sex, age, initial WBC count or the presence of a big tumor mass at diagnosis. There were no significant differences between our two major immunological subclasses Non-T CALLA+ ALL, and Pre-T ALL regarding proportions of patients in continuous remission, and relapse-free survival durations. The analysis of clinical and laboratory characteristics of patients on the basis of their chromosome categories did not show any significant sex bias, age distribution, initial WBC count, tumoral presentation or morphological subtypes at diagnosis, although there was an apparent male predominance in the pseudodiploid category and female predominance in the hyperdiploid category. Our results concerning the prognostic implication of CALLA were contradictory to those of several other investigators.  相似文献   

2.
Multivariate analyses of the clinical course of 1537 children with acute lymphoblastic leukemia (ALL) identified a subgroup which experienced short remission duration and a high incidence of extramedullary relapse. The patients differed from other ALL patients by the presence at diagnosis of two or more of a constellation of clinical and laboratory features: organomegaly or mass disease, E-rosette positivity, hemoglobin level greater than 10 g/dl, leukocyte count greater than 50,000/microliters, male predominance, and older age. This type of presentation of ALL is referred to as the "lymphoma syndrome" (LS) since such patients exhibit a pattern of several clinical and laboratory features which were observed repeatedly but in differing combinations, and some of which clinically resemble lymphoma. A subsequent database from 2231 patients was analyzed. Patients with a mediastinal mass, massive splenomegaly, or massive adenopathy, alone or in combination, had a worse outcome when the patient also had either leukocytosis, E-rosette-positive lymphoblasts, or a normal or near normal hemoglobin (Hb) level at diagnosis. Similarly, the above three laboratory features alone or in combination did not predict less than 40% disease-free survival (DFS) unless they were accompanied by at least one of the clinical features of mass disease. When at least one clinical feature and at least one laboratory feature were present, the overall DFS was 36% 6 years after diagnosis versus 64% for all other patients. The association of these features with poor prognosis remained significant after adjusting for the level of leukocyte count at diagnosis, age at diagnosis, and sex of the patients. Patients with this recurrent syndrome of features do not represent a homogeneous biologic entity but they constitute a subgroup of patients with ALL having a high risk of treatment failure using current therapies, including failure to achieve remission, early relapse, and increased frequency of relapse in extramedullary sites. They deserve early recognition at diagnosis and selection of treatment strategies appropriate for very high risk ALL.  相似文献   

3.
Smoking adversely affects hematopoietic stem cell transplantation outcome. We asked whether smoking affected outcome of newly diagnosed acute myeloid leukemia (AML) patients treated with chemotherapy. Data were collected on 280 AML patients treated with high-dose cytarabine and idarubicin-containing regimens at Roswell Park Cancer Institute who had smoking status data at diagnosis. Patients' gender, age, AML presentation (de novo vs. secondary), white blood cell (WBC) count at diagnosis, karyotype and smoking status (never vs. ever) were analyzed. Among the 161 males and 119 females with a median follow-up of 12.9 months, 101 (36.1%) had never smoked and 179 (63.9%) were ever smokers. The proportion of patients between never and ever smokers was similar to respect to age, AML presentation, WBC count at diagnosis or karyotype based on univariate analysis of these categorical variables. Never smokers had a significantly longer overall survival (OS) (60.32 months) compared to ever smokers (30.89; p = 0.005). In multivariate analysis incorporating gender, age, AML presentation, WBC count, karyotype and smoking status as covariates, age, karyotype and smoking status retained prognostic value for OS. In summary, cigarette smoking has a deleterious effect on OS in AML.  相似文献   

4.
The pretreatment characteristics of 158 children with previously untreated acute lymphoblastic leukemia diagnosed April 1972 to June 1978 were analyzed for their ability to predict prognosis. The children were treated according to therapeutic protocols 721, 745 and 765, by members of the Japanese Children's Cancer and Leukemia Study Group. Multivariate analysis was performed to determined the relationship between the characteristics and duration of survival of the patients. The following characteristics were analyzed: initial white blood cell (WBC) count, age at diagnosis, initial hemoglobin level, initial platelet count, sex, organomegaly, and treatment regimen that was provided. By using multivariate techniques, factors were found which the independently and significantly predict the length of survival. These factors were initial WBC count (r0 = 0.2908), age at diagnosis (r0 = 0.2982), and treatment regimen (r0 = 0.2488). Using the major prognostic factors of age at diagnosis and initial WBC count, a formula to predict the survival time was established. According to the initial WBC count and age at diagnosis, we classified all cases of childhood ALL as standard risk and high risk.  相似文献   

5.
Truong TH  Beyene J  Hitzler J  Abla O  Maloney AM  Weitzman S  Sung L 《Cancer》2007,110(8):1832-1839
BACKGROUND: Tumor lysis syndrome (TLS) is a well-recognized complication of acute lymphoblastic leukemia (ALL). The ability to predict children at differing risk of TLS would be an early step toward risk-based approaches. The objectives of the current study were 1) to describe the prevalence and predictors of TLS in childhood ALL and 2) to develop a sensitive prediction rule to identify patients at lower risk of TLS. METHODS: Health records of children aged /=2 laboratory abnormalities occurring in the time frame of interest. Predictors of TLS were determined using univariate and multiple logistic regression analyses. RESULTS: Among 328 patients, 23% met criteria for TLS. Factors predictive of TLS were male sex (odds ratio [OR], 1.8; P = .041), age >/=10 years (OR, 4.5; P < .0001), splenomegaly (OR, 3.3; P < .0001), mediastinal mass (OR, 12.2; P < .0001), T-cell phenotype (OR, 8.2; P < .0001), central nervous system involvement (OR, 2.8; P = .026), lactate dehydrogenase >/=2000 U/L (OR, 7.6; P < .0001), and white blood count (WBC) >/=20 x 10(9)/L (OR, 4.7; P < .0001). Among variables that were available at presentation, multiple regression analysis identified age >/=10 years, splenomegaly, mediastinal mass, and initial WBC >/=20 x 10(9)/L as independent predictors of TLS. When all 4 of those predictors were absent at presentation (n = 114 patients), the negative predictive value of developing TLS was 97%, with a sensitivity of 95%. CONCLUSIONS: Clinical and laboratory features at the time of presentation identified a group of children with ALL at low risk for TLS that may benefit from a risk-stratified approach directed at reduced TLS monitoring and prophylaxis.  相似文献   

6.
Fms-like tyrosine kinase 3 (FLT3) performs a vital role in the pathogenesis of hematopoietic malignancies.Therefore in recent times, the focus of several studies was on use of FLT3 as a prognostic marker. The presentstudy investigated the molecular characterization and incidence of FLT3 mutations in acute leukemia patients inPakistan. A total of 55 patients were studied, of which 25 were suffering from acute lymphoblastic leukemia (ALL)and 30 were suffering from acute myeloid leukemia (AML). The polymerase chain reaction demonstrated FLT3/ITD mutations in 1 (4%) of 25 ALL patients, a male with the L2 subtype. In AML cases the rate was 4 (13.3%) of30, three males and one female. The AML-M4 subtype was found in three and the AML M2 subtype in the other.In the AML cases, a statistically significant (p=0.009) relationship was found between WBC (109/L) and FLT3/ITD positivity. However, no significant relationship was found with other clinical parameters (p>0.05). In acutemyeloid leukemia (AML) FLT3/ITD+ mutation was more prevalent in elderly patients 31-40 age groups, 21-30and 51-60 age groups respectively. In acute lymphoblastic leukemia (ALL) statistically no significant relationshipwas found between clinical features and FLT3/ITD positivity (p>0.05). However, in acute lymphoblastic leukemia(ALL) FLT3/ITD+ mutation was more commonly found in age groups of 21-30.  相似文献   

7.
Improvements in therapy for childhood acute lymphoblastic leukemia (ALL) have led us to reevaluate the prognostic significance of lymphoblast characteristics at diagnosis. From application of univariate and multivariate statistical methods, we determined the relationship of five blast cell features to treatment outcome in 250 patients who were enrolled in two clinical trials at this center from May 1979 through April 1982. Karyotype ploidy, lymphoblast morphology, and immunophenotype were each significantly related to prognosis as measured by time to failure, while periodic acid-Schiff reactivity and glucocorticoid receptor number lacked prognostic implication for this patient population. In addition, clinical features of initial WBC count, age, and race were also significant independent variables in predicting treatment response. By multivariate analysis, both ploidy and morphology contributed prognostic information to a clinical model based on WBC count, age, and race. If the model was adjusted for impact of ploidy, however, French-American-British morphology no longer contributed additional prognostic information. Our findings suggest that many traditional biological features used to estimate prognosis in ALL can be discarded in favor of clinical features (leukocyte count, age, and race) and cytogenetics (ploidy) for planning of future clinical trials.  相似文献   

8.
Leukemic cells from 124 acute lymphoblastic leukemia (ALL) and 31 chronic lymphatic leukemia (CLL) were examined for sheep erythrocyte receptor (E), surface immunoglobulin (SIg) and their reactivity with a panel of monoclonal antibodies recognizing specific surface antigens including pan-T, Common ALL and Ia antigens. In acute lymphatic leukemia, 33% of patients reveal T-cell receptor associated with higher age group, mediastinal mass and high WBC count. Common ALL was predominant between 2 and 9-yr age group. Among chronic lymphatic leukemia, 2 patients were found to be T-CLL while 29 revealed presence of SIg. Ia antigen was detected in 44.4% of ALL and 64% fo CLL patients. The pattern of surface marker observed in our series may be related to our life style, socio-economic and environmental factors.  相似文献   

9.
PURPOSE: Investigation of minimal residual disease (MRD) in childhood acute lymphoblastic leukemia (ALL) using molecular markers has proven superior to other standard criteria (age, sex, and WBC) in distinguishing patients at high, intermediate, and low risk of relapse. The aim of our study was to determine whether MRD investigation is valuable in predicting outcome in Philadelphia-negative adult patients with ALL. PATIENTS AND METHODS: MRD was assessed in 85 adult patients with B-lineage ALL by semiquantitative immunoglobulin H gene analysis on bone marrow samples collected during four time bands in the first 24 months of treatment. Fifty patients received chemotherapy only and 35 patients received allogeneic (n = 19) or autologous (n = 16) bone marrow transplantation (BMT) in first clinical remission. The relationship between MRD status and clinical outcome was investigated and compared with age, sex, immunophenotype, and presenting WBC count. RESULTS: Fisher's exact test established a statistically significant concordance between MRD results and clinical outcome at all times. Disease-free survival (DFS) rates for MRD-positive and -negative patients and log-rank testing established that MRD positivity was associated with increased relapse rates at all times (P <.05) but was most significant at 3 to 5 months after induction and beyond. MRD status after allogeneic BMT rather than before was found to be an important predictor of outcome in 19 adult patients with ALL tested. In patients receiving autologous BMT (n = 16), the MRD status before BMT was more significant (P =.005). CONCLUSION: The association of MRD test results and DFS was independent of and greater than other standard predictors of outcome and is therefore important in determining treatment for individual patients.  相似文献   

10.
Acute lymphocytic leukaemia (ALL) is heterogeneous in clinical characteristics and immunological properties. Standard surface marker analysis has enabled us to subclassify 121 cases of ALL into four subtypes, i.e. T-ALL, common ALL, null ALL and B-ALL. We have also tried to correlate these subtypes with their clinical characteristics. Our patients had younger ages with a mean age of 13.75. A slight male predominance was observed. There were consistently higher incidences in northern Taiwan in each subtype, but no significant differences in incidences between rural and urban areas. Although there were high incidences of L1 type cell in each immunological subtype, there was no correlation between FAB classification and each subtype, nor did morphologic features relate to cellular origins. Clinical manifestations revealed significantly high incidence of CNS involvement and thymic mass in T-ALL. Hepatosplenomegaly was more common and complete remission rate was higher in children with ALL than in adults.  相似文献   

11.
Many cutpoints have been proposed to categorize continuous variables in childhood acute lymphoblastic leukaemia (white blood cell count, peripheral blast cell count, haemoglobin level, platelet count and age), and have been used to define therapeutic subgroups. This variation in the choice of cutpoints leads to a bias called the 'Will Rogers phenomenon'. The aim of this study was to analyse variations in the relative risk of relapse or death as a function of continuous prognostic variables in childhood ALL and to discuss the choice of cutpoints. We studied a population of 1545 children with ALL enrolled in three consecutive protocols named FRALLE 83, FRALLE 87 and FRALLE 89. We estimated the risk of relapse or death associated with different values of each continuous prognostic variable by dividing the sample into quintiles of the distribution of the variables. As regards age, a category of children under 1 year of age was distinguished and the rest of the population was divided into quintiles. The floated variance method was used to calculate the confidence interval of each relative risk, including the reference category. The relation between the quantitative prognostic factors and the risk was monotonic for each variable, except for age. For the white blood cell count (WBC), the relation is log linear. The risk associated with WBC values in the upper quintile was 1.9 times higher than that in the lower quintile. The peripheral blast cell count correlated strongly with WBC (correlation coefficient: 0.99). The risk increased with the haemoglobin level, and the risk in the upper quintile was 1.3 times higher than that in the lower quintile. The risk decreased as the platelet count increased: the risk in the lower quintile was 1.2 times higher than that in the upper quintile. The risk increased gradually with increasing age above one year. The small subgroup of patients (2.5% of the population) under 1 year of age at diagnosis had a risk 2.6 times higher than the reference category of patients between 3 and 4.3 years of age. When the risk associated with a quantitative prognostic factor varies monotonously, the selection of a cutpoint is arbitrary and represents a loss of information. Despite this loss of information, such arbitrary categorization may be necessary to define therapeutic stratification. In that case, consensus cutpoints must be defined if one wants to avoid the Will Rogers phenomenon. The cutpoints proposed by the Rome workshop and the NCI are arbitrary, but may represent an acceptable convention.  相似文献   

12.
The percentage of total 125I-labeled insulin specifically bound to lymphoblasts was measured in 46 children with leukemia. Among 35 children with newly diagnosed acute lymphoblastic leukemia (ALL), specific insulin binding ranged from 0.09 to 14.8% per 106 blasts. A lower level of insulin binding was correlated with T-cell surface markers (P<0.003), higher hemoglobin level (P<0.005), presence of a mediastinal mass (P<0.01), lower glucocorticoid receptor level (P<0.02), higher platelet count (P<0.04), age < 2 or > 10 yr (P<0.05), white blood cell count ≥ 100 × 103/mm3 (P<0.06) and higher labeling index (P<0.07). It was not correlated with the presence of central-nervous-system disease, FAB classification, or sex. With a follow-up of 24 to 33+ months, insulin binding was not correlated with treatment outcome. Six patients with relapsed ALL and three with acute nonlymphoblastic leukemia showed insulin binding levels similar to those in newly diagnosed ALL patients. Blasts from one patient with B-cell ALL and one with chronic myelogenous leukemia were characterized by lower insulin binding, while lymphoblasts from a patient with T-cell lymphoma bound insulin at marginally detectable levels. In vitro studies with IM-9, NALM-1 and NALM-16 cell lines showed that changes in insulin binding caused by dexamethasone treatment were not correlated with hormone-induced cell death. Although study of insulin binding by malignant lymphoid cells may be important in understanding the biology of leukemic cells, it does not appear to have any obvious clinical utility.  相似文献   

13.
Objective: To compare the clinical and biologic characteristics, response to therapy and outcome of adult patients with ALL above and below the age of 55. Design: Retrospective review of clinical and laboratory data. Setting: University affiliated Cancer Center and Veteran's Hospital. Patients: Thirty-three newly diagnosed, consecutive, adults with ALL seen over a nine-year period. Results: while no differences were demonstrated in the distribution of recognized prognostic indicators (such as cytogenetic abnormalities or immunophenotype), individuals over the age of 55 had significantly lower remission rates and shorter survivals. Conclusions: The outcome of elderly patients with ALL is very poor. This is primarily related to an increase in the number of early deaths during induction, as well as a higher prevalence of disease refractory to standard chemotherapy programmes. There is a need for new treatment protocols designed for the elderly ALL patient, as well as a better understanding of the unique biological characteristics of the disease in this age group.  相似文献   

14.
Background: Leukemia and lymphomas are still the common childhood cancers in Iran. This study was undertaken to determine the prevalence of signs and symptoms of these malignancies in children of Fars Province, Southern Iran. Methods: A total of 368 cases of children who were less than 15 years old and diagnosed as acute lymphocytic leukemia (ALL, n = 211), acute myeloid leukemia (AML, n = 64), Burkitt lymphoma (BL, n = 40), chronic myeloid leukemia (CML, n = 5), Hodgkin's disease (HD, n = 33) or non-Burkitt-type, non-Hodgkin's lymphoma (NBNHL, n = 15) referring to the hospitals of Shiraz University of Medical Sciences from April 1997 to March 2002 were enrolled. A questionnaire was provided to record the age, median age at the onset of the disease, sex, type of malignancy and the signs and symptoms at the time of presentation. Results: The common sign or symptoms were fever (74%), in ALL, AML, NHL, and BL patients, hepatosplenomegaly (100%) in CML patients, and lymphadenopathy (54%) and fever (54%) in Hodgkin's disease. Conclusion: Knowledge of signs and symptoms and types of presentations of childhood leukemia and lymphoma may help a physician to improve the patient's outcome. This study revealed that attention to uncommon signs and symptoms in history taking and physical examination together with laboratory tests may increase the physicians’ awareness and better diagnosis of pediatric malignancies and would also be beneficial for the patient.  相似文献   

15.
为研究P53基因突变与人类白血病的关系,应用免疫组化方法对45例不同类型的白血病标本进行了P53蛋白的检测。结果发现P53蛋白表达阳性率为24.4%,其中在急生髓系白血病为18.2%,而在急性淋系白血病中为27.3%,急性髓,淋两系白现间P53蛋白的表达差异无显著性,P53蛋白的表达量与病人年龄,外周血血红蛋白水平,白细胞计数,血小板计数间也无显著性相关。  相似文献   

16.
Previous studies have demonstrated that TP53 mutation is correlated with insufficient therapy response and unfavorable prognosis in acute lymphoblastic leukemia (ALL). Few studies have investigated the impact of TP53 mutation in ALL patients after haploidentical hematopoietic stem cell transplantation (haplo-HSCT). We completed a retrospective study of 65 ALL patients with available TP53 status who underwent haplo-HSCT. They were divided into a TP53 mutation group (TP53mut) and a TP53 wild-type (TP53wt) group. TP53mut showed comparable 2-year cumulative incidence of relapse (CIR) rates (13.1% vs 12.5%, P = .96) and 2-year leukemia-free survival (LFS) (74.2% vs 77.4%, P = .80) with TP53wt. No significant differences in 2-year overall survival (OS) rates (82.9% vs 87.3%, P = .61) or 2-year NRM rates (12.7% vs 10.2%, P = .69) were observed in TP53mut and TP53wt patients. Multivariate analysis suggested that white blood cell (WBC) count at initial diagnosis (>50 × 109/L: hazard ratio [HR] = 3.860, P = .016) and age (>40 years old: HR = 4.120, P = .012) are independent risk factors for 2-year LFS. Our study showed that TP53 mutations may not be related to the unfavorable impact on survival in ALL patients after treatment with haplo-HSCT. The present results suggested that haplo-HSCT may eliminate the poor prognosis effect of TP53 mutation in ALL.  相似文献   

17.
The presence of a chromosomal translocation in the leukemic cells at diagnosis of acute lymphoblastic leukemia (ALL) in children is associated with a high risk for treatment failure. We have reexamined the relationship between translocations and prognosis in 146 children with ALL who received risk-based therapy such that high-risk patients were treated with intensive drug schedules. In univariate analysis, multiple factors were associated with a relatively poor event-free survival (EFS) including age less than 2 years or greater than 10 years (combined group), WBC count greater than 10 x 10(9)/L, French-American-British (FAB) morphologic classification L2, absence of common ALL antigen (CALLA, CD10) expression, absence of hyperdiploidy with a chromosome number of 50 to 60, and presence of the specific translocations t(4; 11)(q21;q23) or t(9;22)(q34;q11) (combined group). However, there was no disadvantage with respect to EFS in patients with translocations compared with those who lacked translocations (73% at 4 years in both groups). Furthermore, when patients with specific cytogenetic abnormalities for which the prognostic significance has been well established (hyperdiploid 50 to 60, t(4;11), and t(9;22] were removed from the analysis, the remaining group with other translocations had a better EFS than the remaining group lacking translocations, although this was not statistically significant (81% v 65% at 4 years, P = .24). In a multivariate analysis, a model including WBC count and FAB classification was the strongest predictor of EFS. The presence or absence of translocations was not an independent predictor of EFS and did not contribute to the ability of any model to predict EFS. In conclusion, when effective intensive therapy is used to treat childhood ALL with high-risk clinical features, categorization of patients on the basis of chromosomal translocations without attention to the specific abnormality is not useful as a prognostic factor.  相似文献   

18.
The clinical hematologic and pathologic findings of 30 patients with hairy cell leukemia observed between 1966 and 1979 were studied. Twelve patients had long-lasting course of the disease. Seven of them displayed a survival greater than or equal to 120 months, whereas 18 patients died within 36 months of the diagnosis. Their clinical and laboratory characteristics (age of onset, sex, ESR, hemoglobin, WBC, neutrophils, monocytes, platelets, spleen and liver size) were analyzed to ascertain possible prognostic features. Multivariate discriminant analysis, performed both with a direct method and with a stepwise method (Wilks' method), provided a discriminant function able to correctly predict the prognosis of the disease in 83.3% of the examined cases. Spleen size, neutrophil count, age of onset, ESR and liver size turned out to be the most important prognostic factors; in contrast, splenectomy did not significantly affect the prognosis in our cases.  相似文献   

19.
The pretreatment characteristics of 158 children with previouslyuntreated acute lymphoblastic leukemia diagnosed April 1972to June 1978 were analyzed for their ability to predict prognosis.The children were treated according to therapeutic protocols721, 745 and 765, by members of the Japanese Children's Cancerand Leukemia Study Group. A univariate analysis was performed to determine the relationshipbetween the characteristics and the duration of the patients'survival. The following characteristics were analyzed: initialwhite blood cell (WBC) count, age at diagnosis, initial hemoglobinlevel, initial platelet count, sex, organomegaly. and treatmentregimen that was provided. Favorable prognosis was exhibited only by those patients withinitial WBC counts of less than 50,000/mm3, with age at onsetbetween 2 and 6 years, without splenomegaly, and with hemoglobinlevels between 5 and 10 g/dl. The most significant contributions among the various individualprognostic factors were initial WBC count (p < 0.001) andthe age at diagnosis (p < 0.01).  相似文献   

20.
 目的 探讨急性淋巴细胞白血病(ALL)患者DNA拓扑异构酶(Topo)mRNA水平的表达及其临床意义。方法 用半定量反转录-聚合酶链反应(RT-PCR)技术检测了90例ALL患者DNA Topo的表达。结果 DNA Topo各亚型(Topo-Ⅰ,Topo-Ⅱa,Topo-Ⅱb)的表达阳性率初治组(58.1 %,51.2 %,81.5 %)高于复发难治组(33.3 %,44.4 %,77.9 %)和完全缓解(CR)组(14.9 %,23.4 %,34.1 %),各组比较差异均有统计学意义(P<0.05)。表达量各组间比较,CR组与初治组差异有统计学意义(P<0.005);CR组与复发难治组Topo-Ⅱb差异有统计学意义(P<0.05),DNA To各亚型表达量之间有显著相关性(P<0.001),且各亚型表达量与WBC数之间亦有显著相关性(P<0.01)。结论 ALL患者DNA Topo各亚型mRNA水平的表达量可能与WBC数共同作为Topo抑制剂药物个体化合理用药的参考指标,但不宜作为判断患者是否对Topo抑制剂耐药的参考指标。  相似文献   

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