bluuebunny/crossref_metadata_2025_split huggingface dataset:
Quality-of-Care Indicators for Early-Stage Prostate Cancer
Authors: Spencer Benjamin A. , Steinberg Michael , Malin Jennifer , Adams John , Litwin Mark S. 
Published: May 2003

Purpose: Decisions regarding treatment for early-stage prostate cancer are frustrated not only by inadequate evidence favoring one treatment modality but also by the absence of data comparing quality among providers. In fact, the choice of provider may be as important as the choice of treatment. We undertook this study to develop an infrastructure to evaluate variations in quality of care for men with early-stage prostate cancer. Methods: We enlisted several sources to develop a list of proposed quality-of-care indicators and covariates. After an extensive structured literature review and a series of focus groups with patients and their spouses, we conducted structured interviews with national academic leaders in prostate cancer treatment. We then convened an expert panel using the RAND consensus method to discuss and rate the validity and feasibility of the proposed quality indicators and covariates. Results: The panel endorsed 49 quality-of-care indicators and 14 covariates, which make up our final list of candidate measures. Several domains of quality are represented in the selected indicators, including patient volume, pretreatment referrals, preoperative testing, interpretation of pathology specimens, and 10-year disease-free survival. Covariates include measures of case-mix, such as patient age and comorbidity. Conclusion: This study establishes a foundation on which to build quality-of-care assessment tools to evaluate the treatment of early-stage prostate cancer. The next step is to field-test the indicators for feasibility, reliability, validity, and clinical utility in a population-based sample. This work will begin to inform medical decision-making for patients and their physicians.
Adiposity and Sex Hormones in Postmenopausal Breast Cancer Survivors
Authors: McTiernan Anne , Rajan Kumar B. , Tworoger Shelley S. , Irwin Melinda , Bernstein Leslie , Baumgartner Richard , Gilliland Frank , Stanczyk Frank Z. , Yasui Yutaka , Ballard-Barbash Rachel 
Published: May 2003

Purpose: Overweight and obese women with breast cancer have poorer survival compared with thinner women. One possible reason is that breast cancer survivors with higher degrees of adiposity have higher concentrations of tumor-promoting hormones. This study examined the association between adiposity and concentrations of estrogens, androgens, and sex hormone–binding globulin (SHBG) in a population-based sample of postmenopausal women with breast cancer. Methods: We studied the associations between body mass index (BMI), body fat mass, and percent body fat, measured by dual-energy x-ray absorptiometry scan, waist circumference, and waist-to-hip circumference ratio, with concentrations of estrone, estradiol, testosterone, SHBG, dehydroepiandrosterone sulfate, free estradiol, and free testosterone in 505 postmenopausal women in western Washington and New Mexico with incident stage 0 to IIIA breast cancer. Blood and adiposity measurements were performed between 4 and 12 months after diagnosis. Results: Obese women (BMI ≥ 30) had 35% higher concentrations of estrone and 130% higher concentrations of estradiol compared with lighter-weight women (BMI < 22.0; P = .005 and .002, respectively). Similar associations were observed for body fat mass, percent body fat, and waist circumference. Testosterone concentrations also increased with increasing levels of adiposity (P = .0001). Concentrations of free estradiol and free testosterone were two to three times greater in overweight and obese women compared with lighter-weight women (P = .0001). Conclusion: These data provide information about potential hormonal explanations for the association between adiposity and breast cancer prognosis. These sex hormones may be useful biomarkers for weight loss intervention studies in women with breast cancer.
Combination of Fludarabine and Mitoxantrone in Untreated Stages III and IV Low-Grade Lymphoma: S9501
Authors: Velasquez William S. , Lew Danika , Grogan Thomas M. , Spiridonidis C. Harris , Balcerzak Stanley P. , Dakhil Shaker R. , Miller Thomas P. , Lanier Keith S. , Chapman Robert A. , Fisher Richard I. 
Published: May 2003

Purpose: To determine the efficacy of combination fludarabine and mitoxantrone (FN) in untreated stages III and IV low-grade lymphoma. The major end point was to estimate progression-free survival (PFS) in all eligible patients. Patients and Methods: Seventy-eight eligible patients were registered. Chemotherapy courses were administered every 4 weeks with mitoxantrone 10 mg/m2 on day 1 and fludarabine 25 mg/m2 on days 1, 2, and 3 for a total of six to eight cycles. Pneumocystis carinii prophylaxis was required. Results: Seventy-three patients (94%) attained an objective response. Complete remission was demonstrated in 34 patients (44%) and partial remission was demonstrated in 39 patients (50%). With a median follow-up time of 5.5 years, the median PFS was 32 months, with a 4-year PFS rate of 38%. Median survival has not been reached and 88% of all patients are alive at 4 years. The application of the International Prognostic Index and serologic staging showed significant differences in PFS in all risk groups, whereas overall survival was markedly worse for the highest-risk group in either prognostic model. Three prior Southwest Oncology Group trials using a regimen of cyclophosphamide, doxorubicin, vincristine, and prednisone or a combination of prednisone, vincristine, methotrexate, cytarabine, cyclophosphamide, etoposide, nitrogen mustard, vincristine, procarbazine, and prednisone in similar patient populations demonstrated comparable clinical outcome, although the 4-year survival for FN was better. FN was well tolerated, but mild to severe reversible myelosuppression was noted. Other complications were rare. Conclusion: FN is an effective, safe chemotherapy combination for patients with advanced-stage, low-grade lymphoma. Clinical outcomes were comparable to prior published data using anthracycline-based regimens.
Biology, clinical, and hematologic features of acute megakaryoblastic leukemia in children
Authors: Paredes‐Aguilera Rogelio , Romero‐Guzman Lina , Lopez‐Santiago Norma , Trejo Rosa Arana 
Published: May 2003

AbstractTo assess the incidence, clinical features at presentation, hematologic, immunophenotypic, and cytogenetic characteristics of AMKL in children we prospectively studied 834 consecutive non selected children with newly diagnosed acute leukemia (AL) admitted to the Hematology Department at the Instituto Nacional de Pediatría (INP), Mexico, D.F. We found 682 cases (81.8%) with a typical ALL immunophenotype, and the remaining 152 (18.2%) were considered to have AML. In 29 of the 152 patients with AML studied, a diagnosis of AMKL was established. These 29 cases represented 19.1% of the cases of AML and 3.48% of the total cases of AL during the time span covered by the study. Twenty‐four percent of the cases occurred in infants 2 years old or younger and 41.4% occurred in children 41 months of age or younger. In contrast, in only 18.6% of the patients with AML (M0–M6), the diagnosis was established before 42 months of age and in 17% before their second year of life. Clinical presentation was not strikingly different than that observed in patients with other types of AML, and the time interval from onset of symptoms to diagnosis was also similar, though in a small subset of patients, the clinical course was characterized by a chronic slowly progressive disorder extending over weeks or months resembling smoldering leukemia or chronic myelofibrosis with agnogenic myeloid metaplasia. Bone marrow (BM) fibrosis was a constant features in our patients; 75% of the patients studied showed this complication at the time of diagnosis. Some rather unusual findings in this study were intense skeletal pains from multiple osteolytic lesions, the presence of soft‐tissue tumor, and the presence of cohesive scanty clusters of primitive‐looking blast cells in BM aspirates. Several interesting cytogenetic findings in our study were t(1;22)(p13;q13) in a 14‐year‐old boy, t(9;22)(q34;q11) in one patient, and monosomy 7 in two patients. Another important finding in our study was the clinical association with colonic adenocarcinoma in one patient, an association that to our knowledge has not been reported previously. In conclusion, our data suggest that the incidence of AMKL in Mexico might be higher than those reported in Caucasian white pediatric population, and that biologic and cytogenetic profile may differ from those of western countries, but more studies are needed to corroborate cytogenetic heterogeneity, ethnic and geographic diversity. Early onset of the disease, low WBC counts, slight thrombocytopenia or normal platelet counts, and BM fibrosis were characteristic distinctive features of at least half of the patients with this subtype of AML. Am. J. Hematol. 73:71–80, 2003. © 2003 Wiley‐Liss, Inc.
Intravenous immunoglobulin therapy results in post‐infusional hyperproteinemia, increased serum viscosity, and pseudohyponatremia
Authors: Steinberger Beth A. , Ford Stephen M. , Coleman Teresa A. 
Published: May 2003

AbstractIntravenous immunoglobulin (IVIG) therapy is associated with rare reports of thromboembolic events and severe hyponatremia. We hypothesized that IVIG therapy may result in hyperproteinemia, increased serum viscosity, and pseudohyponatremia. We conducted a prospective observational study to evaluate the incidence of hyperproteinemia occurring after IVIG therapy and its relationship to serum sodium, viscosity, osmolality, and the serum osmolar gap. Eighteen IVIG infusions at a standard dose of 2 g/kg administered over 2–5 days were evaluated. Serum glucose, sodium, protein, viscosity, osmolality, and a calculated osmolar gap were obtained prior to therapy, 6 hr after the initiation of therapy, 24 hr after the conclusion of therapy, and on post‐treatment day 10. Paired t‐testing revealed a statistically significant increase in serum protein and viscosity and decrease in serum sodium and calculated osmolality 24 hr after the completion of IVIG therapy. The calculated serum osmolar gap increased insignificantly. In multivariate analysis, hyperproteinemia at the 6‐hr time point predicted hyponatremia (P < 0.000), and hyperproteinemia at the 24‐hr time point predicted both hyponatremia and increased serum viscosity (P = 0.024). These data demonstrate that increased serum viscosity occurs following IVIG therapy due to hyperproteinemia, and the rare hyponatremia reported is a pseudohyponatremia also due to hyperproteinemia. Am. J. Hematol. 73:97–100, 2003. Published 2003 Wiley‐Liss, Inc.
Factor V leiden and prothrombin gene G20210A mutation in children with cerebral thromboembolism
Authors: Bonduel Mariana , Sciuccati Gabriela , Hepner Mirta , Pieroni Graciela , Torres Aurora Feliú , Mardaraz Claudia , Frontroth Juan Pablo 
Published: May 2003

AbstractWe investigated whether there is an association between factor V Leiden (FVL) and/or prothrombin gene G20210A mutation (PT20210A) and cerebral thromboembolism in a pediatric Argentinean population. From May 1992 to January 2002, 44 consecutive children with arterial ischemic stroke (AIS) and 23 children with cerebral sinovenous thrombosis (SVT) were prospectively studied at a single center. The prevalence of both mutations was compared with a 102 age‐matched controls. In children with AIS, the frequencies (patients vs. controls), odds ratio (OR), and 95% confidence interval (95% CI) for the presence of FVL were as follows: 2.3% vs. 2%, OR/95% CI, 1.16/0.2 to 13.2; P value = 0.99. No cases of PT20210A were found in this group. In children with SVT, the frequencies (patients vs. controls), OR, and 95% CI were as follows: FVL (4.3% vs. 2%, OR/95% CI, 2.27/0.22 to 6.2; P value = 0.99) and PT20210A (4.3% vs. 1%; OR/95% CI, 4.6/0.3 to 76.3; P value = 0.3354). One child with PT20210A also had an inherited protein C deficiency. In 12 (18%) out of the 67 children with cerebral thromboembolism, without the aforementioned mutations, other prothrombotic disorders were detected. Although a multi‐center prospective study with a large number of Argentinean pediatric patients is needed to obtain considerable evidence, no association between factor V Leiden and/or prothrombin gene G20210A mutation and cerebral thromboembolism was found in this pediatric series. Am. J. Hematol. 73:81–86, 2003. © 2003 Wiley‐Liss, Inc.
Health status and health‐related quality of life associated with von Willebrand disease
Authors: Barr Ronald D. , Sek Julia , Horsman John , Furlong William , Saleh Mahassen , Pai Mohan , Walker Irwin 
Published: May 2003

AbstractVon Willebrand disease (VWD) is the commonest inherited disorder of hemostasis and the majority of women with this disorder experience excessive uterine bleeding. Yet very little information is available on the health‐related quality of life (HRQL) in individuals with VWD. To test the a priori hypotheses that these individuals will have poorer HRQL than members of the general population, and that this burden of morbidity will correlate with the severity of VWD, a cross‐sectional study was undertaken of a population‐based cohort in a regional hemophilia program in Ontario, Canada. A survey was made of individuals over 13 years of age with VWD who self‐reported their health status using a standard 15 item questionnaire. The responses were converted to levels in the Health Utilities Index Mark 2 (HUI2) and Mark 3 (HUI3) health status classification systems to form multi‐element vectors from which single attribute morbidity and overall HRQL utility scores were determined. As a group, individuals with VWD were shown to have poorer HRQL than members of the general population and those with Type 2 disease carried a greater burden of overall morbidity than those with Type 1 disorder. Morbidity was evident mainly in the attributes of emotion, cognition with pain. A striking difference was observed between males and females, with the latter having overall HRQL utility scores similar to those reported previously for HIV positive, severe hemophiliacs. It is possible that this remarkable burden of morbidity reflects chronic iron deficiency associated with menorrhagia. A national study has been proposed to address this likelihood as it offers an opportunity for effective therapeutic intervention (iron supplementation) with a concomitant gain in health status and HRQL. Am. J. Hematol. 73:108–114, 2003. © 2003 Wiley‐Liss, Inc.
Complete recovery from refractory immune thrombocytopenic purpura in three patients treated with etanercept
Authors: McMinn J.R. , Cohen Stephen , Moore Joseph , Lilly Scott , Parkhurst Joan , Tarantino Michael D. , Terrell Deirdra R. , George James N. 
Published: May 2003

AbstractManagement of patients with immune thrombocytopenic purpura (ITP) who have persistent, severe, and symptomatic thrombocytopenia following splenectomy is difficult and empirical. No single agent or regimen provides long‐term success for most patients, and for most treatments it is difficult to assess whether benefits outweigh risks. We report three consecutive patients with critical chronic refractory ITP, who responded promptly and completely following treatment with etanercept, an inhibitor of tumor necrosis factor‐alpha. These patients had failed 6–11 previous treatments. In the first patient, etanercept was given for its approved indication: a flare of co‐existing rheumatoid arthritis. The next two patients were treated with etanercept because of successful outcomes in the previous patients. Although etanercept appeared to be effective treatment for ITP in these 3 patients, the experimental nature of this treatment and the potential risks must be emphasized. On the basis of these case reports, a clinical trial has been initiated to systematically evaluate the efficacy and risks of etanercept in the management of children and adults with chronic ITP. Am. J. Hematol. 73:135–140, 2003. © 2003 Wiley‐Liss, Inc.
Clinicopathologic analysis of ocular adnexal lymphomas: Extranodal marginal zone b‐cell lymphoma constitutes the vast majority of ocular lymphomas among Koreans and affects younger patients
Authors: Cho Eun Yoon , Han Jae Joon , Ree Howe Jung , Ko Young Hyeh , Kang Yoon‐koo , Ahn Hyo Sook , Ahn Seung Do , Park Chan Jeoung , Huh Jooryung 
Published: May 2003

AbstractThe majority of ocular adnexal lymphomas (OAL) are primary marginal zone B‐cell lymphomas (MALT lymphomas). The present study correlated the clinicopathological variables with the histologic subtypes by World Health Organization (WHO) classification with emphasis on MALT lymphomas in OALs of Koreans. There were 68 cases (31 males and 37 females), with a mean age of 45.9 years (range 7–89 years). Histologically, 61 MALT‐type, 2 diffuse large B‐cell (DLBCL), 2 mantle cell type (MCL), 1 anaplastic large‐cell (ALCL), and 2 NK/T‐cell lymphomas (NK/T‐L) were counted among them. Fifty‐seven were primary cases (P‐OAL), and 11 were secondary cases (S‐OAL). Nearly all P‐OALs were MALT lymphomas (n = 56, 98%), with an exception of 1 MCL. Eleven S‐OALs included 5 MALT type, 2 DLBCL, 1 ALCL, 1 MCL, and 2 NK/T‐L. All MALT lymphoma patients were alive (n = 59) except for 2 after a mean duration of follow‐up of 27.6 months (range: 0–108 months): one died of an unrelated cause and one died of recurrence. One non‐MALT type P‐OAL was alive with no evidence of disease (42 months). Of the 11 S‐OAL, 4 had marrow involvement and 5 had progression or relapse outside the orbit. Compared with the other subtypes, MALT lymphoma was more likely to present with local disease (P = 0.001), achieve complete remission (CR) (0.022), and be alive at last follow‐up (0.197), and less likely to experience recurrence (P = 0.06). In conclusion, OALs in Koreans are characterized by a preponderance of primary lymphomas over systemic lymphomas, striking predominance of MALT type lymphomas, and young age of occurrence. Histologic subtype by WHO classification has a significant correlation with the final outcome, with the most favorable outcome associated with OALs of the MALT type. Am. J. Hematol. 73:87–96, 2003. © 2003 Wiley‐Liss, Inc.
Prognostic significance of bcl‐2, bax, and p53 expression in diffuse large B‐cell lymphoma
Authors: Sohn Sang Kyun , Jung Jin Tae , Kim Dong Hwan , Kim Jong Gwang , Kwak Eun Kyung , Park Tae in , Shin Dong Gun , Sohn Kyung Rak , Lee Kyu Bo 
Published: May 2003

AbstractDiffuse large B‐cell lymphoma (DLCL) exhibits heterogeneous clinical features and varies markedly in response to treatment and prognosis. Because apoptosis‐related proteins may play an important role in predicting the prognosis of DLCL, the current study investigated the prognostic significance of a high level of bcl‐2, bax, and p53 expression in relation to clinical characteristics in patients with DLCL. Paraffin‐embedded specimens from 94 patients with de novo DLCL were analyzed immunohistochemically for bcl‐2, bax, and p53 gene expression. Cases with a positive immunohistological stain in more than 50% of the tumor cells were considered to have DLCL‐positive expression. Patients were treated optimally, i.e., with radiotherapy including brief cycles of CHOP or CHOP‐like regimens for patients with stage 1–2A diseases and with at least 6 cycles of CHOP or CHOP‐like regimens for stage 2B–4 diseases. The responses to therapy and survival were then analyzed in 94 uniformly staged patients. bcl‐2 expression was identified in 24 patients (26.4%), bax expression in 35 patients (37.6 %), and p53 expression in 21 patients (22.6%). bax expression proved to be a statistically significant prognostic factor in predicting the overall survival (OS) (P = 0.0015) and disease‐free survival (DFS) (P = 0.0052), regardless of other clinical factors or immunohistological results. There was no significant difference in the OS (P = 0.0682) or DFS (P = 0.088) between the bcl‐2‐positive (n = 24) and bcl‐2‐negative (n = 67) groups. However, bcl‐2 expression was found to be unfavorably associated with the OS (P = 0.0054) in a confined group with low (n = 51) or low intermediate (n = 22) IPI scores. The expression of p53 exhibited no statistical correlation with the OS or DFS. A multivariate analysis revealed that IPI score, bulky mass, and bax expression were all significantly associated with the DFS or OS. bax and bcl‐2 should be considered as independent biologic prognostic parameters in DLCL, thereby aiding in the identification of patient risk groups. As such, bcl‐2‐positive patients with a low or low intermediate IPI score, or without a high level of bax expression could be candidates for more intensive therapy or alternative therapeutic approaches. Am. J. Hematol. 73:101–107, 2003. © 2003 Wiley‐Liss, Inc.
Isolated noncompaction of the left ventricular myocardium in the adult is an autosomal dominant disorder in the majority of patients
Authors: Sasse‐Klaassen Sabine , Gerull Brenda , Oechslin Erwin , Jenni Rolf , Thierfelder Ludwig 
Published: May 2003

AbstractIsolated noncompaction of the ventricular myocardium (INVM, MIM 300183 and 604169) is a congenital unclassified cardiomyopathy with numerous prominent trabeculations and deep intertrabecular recesses in a hypertrophied and hypokinetic myocardium. Mutations in the G4.5 gene result in a wide spectrum of severe infantile X‐linked cardiomyopathic phenotypes including Barth syndrome with dilated cardiomyopathy and INVM. Molecular genetic analysis of INVM has only been performed in pediatric patients. Although adult INVM patients show similar cardiac abnormalities, the influence of genetic factors, especially of mutations in G4.5, is unknown. We analyzed 25 adult INVM patients for the presence of mutations in the G4.5 gene and performed a pedigree analysis of probands. Mutations were not found in the coding sequence or splice sites of G4.5. Systematic analysis of relatives from seven of nine probands showed multiple affected members consistent with an autosomal dominant pattern of inheritance in the majority of cases. We conclude that INVM in the adult is an autosomal dominant disorder rarely caused by mutations in G4.5 and therefore genetically distinct from infantile X‐linked cases. © 2003 Wiley‐Liss, Inc.
Malignant hematological disorders in children with Wolf–Hirschhorn syndrome
Authors: Sharathkumar Anjali , Kirby Melanie , Freedman Melvin , Abdelhaleem Mohamed , Chitayat David , Teshima Ikuko E. , Dror Yigal 
Published: May 2003

AbstractWolf–Hirschhorn syndrome (WHS) is a rare chromosomal disorder attributable to a deletion at the short arm of chromosome 4. This syndrome is associated with characteristic facial appearance, multiple congenital abnormalities, mental retardation, feeding difficulties and failure to thrive. We report two girls with WHS who developed myelodysplastic syndrome (MDS). According to the “Category, Cytology, Cytogenetic (CCC)”classification of childhood MDS, patient 1 had refractory cytopenia with ring sideroblasts at the age of 6 years, while patient 2 had refractory cytopenia with dysplasia at the age of 5½ years. Patient 1 progressed to refractory cytopenia with excess blasts within a year, while patient 2 progressed to acute lymphoblastic leukemia within 1 month of presentation. It is possible that allelic loss of a tumor suppressor gene such as WHSC1 and/or FGFR3 from the deleted segment 4p16.3 plays a critical role in the process of malignant transformation. To our knowledge, this is the first report of severe hematological complications like MDS and leukemia in children with WHS and may be an important genetic model for understanding malignant hematological transformation. This report also underscores the importance of evaluating children with WHS for hematopoietic dysfunction. © 2003 Wiley‐Liss, Inc.