bluuebunny/crossref_metadata_2025_split huggingface dataset:
Malignant Supratentorial Ganglioglioma (Ganglion Cell–Giant Cell Glioblastoma)
Authors: Dash Rajesh C. , Provenzale James M. , McComb Rodney D. , Perry Deborah A. , Longee Darryl C. , McLendon Roger E. 
Published: January 2021

Abstract
Background.—From both epidemiologic and pathologic viewpoints, gangliogliomas exhibiting components of giant cell glioblastomas are extraordinary neoplasms. We report herein the case of a 6-year-old girl who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell tumor–giant cell glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period.
Methods.—Formalin-fixed, paraffin-embedded tissue blocks were sectioned at 5 μm for histochemical and immunohistochemical analyses. Hematoxylin-eosin–stained sections and immunohistochemically stained sections from the primary and secondary resections were reviewed. Reactivity for glial fibrillary acidic protein, neurofilament protein, synaptophysin, and Ki67 nuclear antigen was evaluated.
Results.—Histologically, 2 distinct cell populations were noted on both the primary and secondary resections. The primary resection revealed a neoplasm having a predominant glial component consistent with a glioblastoma. Interspersed were dysmorphic ganglion cells supporting a diagnosis of ganglioglioma. The second resection (following therapy) demonstrated a much more prominent dysmorphic ganglion cell component and a subdued glial component.
Conclusion.—Although immunohistochemical analysis clearly distinguished the 2 tumor cell populations, the identification of Nissl substance in neurons proved to be equally helpful. Although other cases of grade III gangliogliomas and rare cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the only report of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histologic findings in a giant cell glioblastoma and a ganglioglioma and documents the aggressive biologic behavior of this complex neoplasm.
Small Cell Carcinocythemia
Authors: Sile Cynthia C. , Perry David J. , Nam Lucy 
Published: January 2021

Abstract
Tumor cells circulate in the blood, but it is unusual to find cancer cells on a routine peripheral smear. The term carcinocythemia is used to describe this phenomenon. Four patients have been reported previously with small cell lung cancer and carcinocythemia. A fifth case is described in this article. These patients presented with advanced disease and had generalized lymphadenopathy and liver involvement; 3 had splenic metastases. All of these patients experienced rapid clinical deterioration. Death occurred 3 days to 2 weeks from the time carcinocythemia was detected. The presence of small cell carcinocythemia can be one of the manifestations of end-stage disease and is associated with an extremely poor prognosis.
Complex Vascular Lesions at Autopsy in a Patient With Phentermine-Fenfluramine Use and Rapidly Progressing Pulmonary Hypertension
Authors: Strother Jennifer , Fedullo Peter , Yi Eunhee S. , Masliah Eliezer 
Published: January 2021

AbstractAnorectic agents, such as aminorex and fenfluramine derivatives, have been associated previously with the development of primary pulmonary hypertension. The combination diet drug phentermine-fenfluramine (or “phen-fen”) was recently marketed in the United States. We describe a case of a 39-year-old woman with rapidly progressing, fatal pulmonary hypertension following administration of phentermine-fenfluramine. Autopsy was remarkable for complex pulmonary vascular lesions most consistent with thrombotic arteriopathy.
Oncocytic Mucoepidermoid Carcinoma of the Trachea
Authors: López-Terrada Dolores , Bloom M. G. K. , Cagle Philip T. , Ostrowski Mary L. 
Published: January 2021

Abstract
We report a rare case of an oncocytic mucoepidermoid carcinoma of the trachea, which presented in a 78-year-old woman with hemoptysis. Oncocytic cells comprised the majority of this low-grade lesion and demonstrated granular cytoplasmic phosphotungstic acid–hematoxylin staining as well as strong immunohistochemical reactivity to antimitochondrial antibody. Most tracheobronchial tumors with oncocytic change are carcinoid tumors. To our knowledge, this is the first oncocytic mucoepidermoid carcinoma of the trachea reported. This diagnosis was facilitated by histochemical and immunohistochemical studies.
Collecting Duct Meningeal Carcinomatosis
Authors: Olivere Joseph W. , Cina Stephen J. , Rastogi Pawan , Ro Jae Y. 
Published: January 2021

AbstractCollecting duct carcinoma (CDC) is an aggressive primary renal neoplasm that represents a distinct subtype of renal cell carcinoma. Histochemical (eg, mucicarmine) and immunohistochemical (eg, Ulex europaeus) studies, taken in concert with the gross and histologic findings, allow differentiation of CDC from the conventional varieties of renal cell carcinoma in most cases. Collecting duct carcinoma generally pursues a more aggressive course than conventional renal cell carcinoma. Metastases to regional lymph nodes, bone, adrenal glands, lung, and skin have been reported in CDC. We describe the case of a 26-year-old man who presented with a clinical and radiologic impression of multifocal meningioma. Biopsies of the meninges and extracranial soft tissues revealed metastatic adenocarcinoma; subsequent studies suggested metastatic CDC. Ultrasound-guided biopsy was performed on a subsequently identified renal mass, which showed features consistent with CDC. To our knowledge, this is the first reported case of meningeal carcinomatosis due to CDC. The diagnostic features of this tumor are discussed.
The Changing Relationships in Transfusion Medicine
Authors: Sazama Kathleen 
Published: January 2021

Abstract
Maintaining quality in provision of transfusion services in the face of mergers, acquisitions, affiliations, and risk-sharing relationships between organizations that formerly conducted business in a traditional vendor-purchaser model is the ultimate challenge. Publications, both lay and professional, highlight the speed and nature of the impetus for change, especially in the United States, where managed care philosophies are driving a bottom-line mentality. Blood collection and transfusion organizations are developing new relationships, including entry of for-profit entities into a formerly virtually exclusively not-for-profit environment, provision of transfusion services by formerly exclusive blood collection entities and vice versa, outsourcing of selected portions, and other innovative relationships, with significantly more competitive marketing strategies. Measures of quality of transfusion services should benchmark current practices, if possible, before entering into new relationships to ensure that the quality of patient care remains high. Concerns about the fiscal viability of organizations should not minimize safety and availability of blood for transfusion when needed.
Impact of Innovations on Transfusion Medicine
Authors: Sacher Ronald A. , Sandler S. Gerald 
Published: January 2021

Abstract
The final decade of the last century of the second millennium ad has seen dramatic changes in all aspects of science and health care. In transfusion medicine, the blood supply is the safest it has ever been. Newer refinements and innovations are continuously being researched and implemented to achieve and further enhance safety. Advances in blood conservation, pharmacologic manipulation, engineered blood derivatives, and recombinant growth factors can now provide safer and more effective alternatives to blood transfusions for many patients. This overview highlights selective innovations in transfusion medicine and emphasizes some significant advances that have occurred in blood donor screening, blood component collections and therapy, and laboratory testing. Newer technologies are anticipated that will further enhance the safety of blood and transfusions and potentially augment annually the blood supply on a worldwide basis.
Evaluation and Implementation of the Gel Test for Indirect Antiglobulin Testing in a Community Hospital Laboratory
Authors: Cate John C. , Reilly Nancy 
Published: January 2021

Abstract
Background.—The gel test, developed by Lapierre in 1984, was designed to standardize antiglobulin testing while improving sensitivity and specificity of the method.
Principle.—Anti–human serum immunoglobulin G (IgG) mixed with Sephadex G100 (gel phase) in a microtube traps red cell–IgG agglutination complexes during migration through the gel in a centrifugation step. Agglutination complexes are visibly detectable at various levels in the microtube as an inverse function of antibody coated on red cells. Unsensitized red cells form a cell pellet at the base of the microtube.
Objective.—To determine if indirect anti–human globulin testing could be standardized and simplified by replacing the tube test with the gel test without compromising quality or increasing costs.
Setting.—A medium-sized community hospital.
Results.—In a blinded retrospective study, we used patient sera (n = 40), which included 10 positive specimens containing 18 known antibodies. Sixteen antibodies were detected and identified with the tube method (1 anti-D and 1 anti-C not detected). By the gel test, 18 antibodies were detected and identified. All negative samples showed 100% concordance. Favorable results were obtained in a nonblinded prospective correlation study (n = 121). Our technologists found the gel test easier to read and more reproducible and reliable than the tube method; they also found increased sensitivity for detecting weakly reacting antibodies. We successfully introduced the gel test into our laboratory as the standard method for indirect antiglobulin testing. Following implementation, improved personnel management was achieved.
Conclusions.—The gel test is a reliable and advantageous method and is appropriate for routine use for detection and identification of alloantibodies in a community hospital transfusion service laboratory.
Severe Hemolytic Reaction Due to Anti-JK3
Authors: Marshall Carol S. , Dwyre Denis , Eckert Robin , Russell Liisa 
Published: January 2021

Abstract
A 35-year-old gravida 3, para 3 Filipino woman with a negative antibody screen, no prior history of transfusion, and no hemolytic disease of the newborn in her children suffered a massive postpartum hemorrhage requiring transfusion. A severe hemolytic transfusion reaction occurred 5 days after delivery. Subsequently, a panagglutinin on a routine antibody identification panel was identified as anti-Jk3. The patient's red blood cell phenotype was Jk(a−b−) and all of her children were Jk(a−b+), yet the antibody that formed reacted with equal strength against all Jka- or Jkb-positive cells. The rare Jk(a−b−) phenotype is more common in Polynesians. Anti-Jk3, like other Kidd system antibodies, is difficult to detect because in vivo production may be absent between provocative episodes and because these antibodies often show weak in vitro reactions. The increasing numbers of Pacific Islanders in the United States could result in more frequent encounters with this rare phenotype. Increased awareness of ethnic variability in blood phenotypes and of the capricious nature of Kidd antibodies can help pathologists and technologists deal more effectively with these cases.
Infection, Immunity, and Cancer
Authors: Persing David H. , Prendergast Franklyn G. 
Published: January 2021

Abstract
A significant percentage of human cancers worldwide are associated with infections due to known viruses, including human papillomaviruses (cervical cancer and other skin cancers), human T-lymphotropic viruses (adult T-cell leukemias and lymphomas in endemic areas), hepatitis B virus (liver cancer), and Epstein-Barr virus (Burkitt lymphoma and nasopharyngeal carcinoma). The fraction of human cancers attributable to infection may now need to be revised in light of the fact that new viral associations have been discovered and other nonviral associations have been identified. This article addresses the increasingly recognized role of infectious agents as precipitants of human neoplasia and the possibility that novel diagnostic, therapeutic, and chemopreventive strategies may emanate directly from research directed at identifying and understanding these agents.
Cystic Fibrosis
Authors: Grody Wayne W. 
Published: January 2021

Abstract
Objective.—To review the current status of scientific knowledge and opinion regarding molecular genetic testing of mutations in the CFTR gene for purposes of diagnosis and population carrier screening of cystic fibrosis (CF).
Data Sources.—Published research findings on the nature of the CFTR gene, pilot population screening studies in the United States and Europe, and ongoing deliberations of professional and governmental agencies considering implementation of widespread testing.
Study Selection.—Findings relevant to the molecular heterogeneity of CFTR mutations and its implications for population carrier screening were considered.
Data Extraction.—Information was extracted from studies published by us and others, as made available to recent consensus panels and professional committees.
Data Synthesis.—These data were reevaluated in light of recent movements in professional and public policy regarding acceptability and desirability of widespread CF mutation testing. Effects to date of such testing on patient outcomes is reported.
Conclusions.—The ability to test for CFTR mutations at the molecular level has already improved the diagnosis of symptomatic patients and expanded the reproductive options of family members of CF patients. The same technology also holds promise of identifying asymptomatic carriers and at-risk couples without family history in the general population so that they too might be offered prenatal diagnosis or other options. However, a number of key questions remain to be worked out before a widespread national screening program can be put into practice. These include the target population to be offered testing (the entire population vs high-risk ethnic groups), the size and nature of the mutation test panel (universal vs ethnic specific), the inclusion or exclusion of CFTR variants that do not cause classical CF, the optimal testing technology, appropriate standards for laboratory quality assurance, and the development of sufficient educational materials and genetic counseling resources for test delivery, reporting, and interpretation. The answers to these questions will be relevant not only to CF testing but also to many other large-scale molecular genetic screening programs being considered in the future.
Utility of RET Mutation Analysis in Multiple Endocrine Neoplasia Type 2
Authors: Noll Walter W. 
Published: January 2021

Abstract
Objective.—To review the role of RET mutation analysis in the diagnosis of multiple endocrine neoplasia type 2 (MEN 2) and in presymptomatic screening for this disorder.
Data Sources.—Review of the medical literature and current clinical practice.
Conclusions.—RET mutation analysis is a sensitive and specific test for MEN 2. It plays a pivotal role in the diagnosis and management of patients and families with MEN 2 and in the individual who presents with an apparently sporadic medullary thyroid carcinoma or pheochromocytoma. These disorders may first come to the attention of either the anatomic or clinical pathologist, who has the opportunity to see that appropriate testing is done. As with any familial disease, professional genetic counseling is an important part of the care of these patients.
Efforts to Regulate the Collection and Use of Genetic Information
Authors: Reilly Philip R. 
Published: January 2021

Abstract
Public fascination with and support for genetic medicine is complicated by a deeply held fear that genetic information will be used by third parties (eg, insurers, employers, school systems) in ways that will harm the individuals from whom it was derived. Since the mid-1990s there has been much state and some federal legislative activity to address 2 closely related issues: the maintenance of genetic privacy and the prevention of genetic discrimination. These laws have had to confront several challenging questions such as what constitutes a genetic test, is genetic information qualitatively different from other medical information, and is there a means to distinguish between the two. In general the state laws are not well crafted. I will argue that a far more preferable policy is to draft a global, comprehensive medical records privacy law and to develop a model statute that defines the role of predictive genetic information in insurance underwriting. Concerns over misuse of genetic information also pose major issues for the conduct of genomic research. Among those I discuss are ownership of the DNA sample, significant changes in the scope of consent that must precede the decision to volunteer as a subject in genomic research, the reuse of long-archived samples, the challenges to intellectual property rights that flow from research, and the rise of the doctrine of community consent.
Interobserver Variability in Subclassification of Squamous Intraepithelial Lesions
Authors: Woodhouse Sherry L. , Stastny Janet F. , Styer Patricia E. , Kennedy Mary , Praestgaard Amy H. , Davey Diane D. 
Published: January 2021

Abstract
Objective.—To determine whether, on a national cytology proficiency test, a competent cytologist can consistently distinguish grades of squamous intraepithelial lesions.
Design.—Results for low- and high-grade squamous intraepithelial lesion referenced slides from the College of American Pathologists Interlaboratory Comparison Program in Cervicovaginal Cytology for 1996 and 1997 were analyzed including educational, nongraded vs graded validated slides.
Results.—The discrepant rate between low- and high- grade lesions ranged from 9.8% to 15% for cytotechnologist, pathologist, laboratory, and all responses. There was a statistically significant difference in performance on graded, validated slides vs educational slides with better performance on validated slides.
Conclusion.—This significant interobserver variability in subclassification of squamous lesions should be considered in management guidelines for abnormal Papanicolaou test results and implementation of national cytology proficiency testing.
Prostate-Specific Antigen Expression and Lipochrome Pigment Granules in the Differential Diagnosis of Prostatic Adenocarcinoma Versus Seminal Vesicle–Ejaculatory Duct Epithelium
Authors: Shidham Vinod B. , Lindholm Paul F. , Kajdacsy-Balla André , Basir Zainab , George Varghese , Garcia Fernando U. 
Published: January 2021

Abstract
Background.—Lipochrome pigment granules (LPGs) and prostate-specific antigen (PSA) localization have been cited as helpful adjuncts in differentiating atypical histologic patterns of seminal vesicle–ejaculatory duct (SVED) from prostatic adenocarcinoma. However, LPGs have been described in both benign and neoplastic prostatic acini, and PSA expression within the intraprostatic SVED has not been fully explored.
Design.—Fifty radical prostatectomy specimens were studied for LPGs and 9 cases for PSA expression.
Results.—Two morphologic types of LPGs (type 1 and type 2) were observed. The reproducibility in classifying LPGs was evaluated by κ statistics, which demonstrated a strong agreement between 4 observers. Type 1 was restricted to SVED in all 50 specimens. Type 2 was subclassified into 2A and 2B. Type 2 LPGs were observed in prostatic acini of different zones, high-grade prostatic intraepithelial neoplasia, prostatic adenocarcinoma, and occasionally with type 1 LPG in SVED. Focal reactivity for PSA in the distal portion of SVED near urethra was noted in 1 of 9 cases.
Conclusion.—Awareness about morphologic differences between the 2 types of LPGs could help to avoid a potential diagnostic pitfall of misinterpreting SVED epithelium for adenocarcinoma. Caution is recommended in interpreting PSA expression, since rare focal PSA reactivity was observed in the distal SVED.
Evaluation of the Enhanced Amplified Mycobacterium Tuberculosis Direct Test for Direct Detection of Mycobacterium tuberculosis Complex in Respiratory Specimens
Authors: Smith Michael B. , Bergmann John S. , Onoroto Michelle , Mathews Greg , Woods Gail L. 
Published: January 2021

Abstract
Objective.—To evaluate the performance of the enhanced Mycobacterium Tuberculosis Direct Test (E-MTD), for the direct detection of M tuberculosis complex (MTBC) in respiratory specimens.
Design.—Two hundred seventy-four respiratory specimens from 151 patients in respiratory isolation were tested with the E-MTD, and the results were compared with the results of mycobacterial smear, culture, and the earlier form of the test, MTD-1.
Results.—Forty-one specimens were culture positive for mycobacteria (20 MTBC and 21 nontuberculous mycobacteria), 23 of which were smear positive (16 MTBC, 7 nontuberculous mycobacteria). Twenty-four specimens were positive by E-MTD, and 21 were positive by MTD-1. Of the 20 MTBC culture-positive specimens, 19 were positive by the E-MTD and 19 were positive by the MTD-1. The remaining specimens were MTBC negative by all methods. After resolution of discrepancies, the sensitivity, specificity, and positive and negative predictive values were 95.2%, 100%, 100%, 99.6% for the MTD-1 and 95.2%, 98.8%, 87.0%, and 99.6%, for the E-MTD. For the E-MTD smear-positive and smear-negative specimens, these same values were 93.8%, 100%, 100%, and 87.5% and 100%, 98.8%, 62.5%, and 100%, respectively.
Conclusion.—The results suggest that the E-MTD is a reliable method for the direct detection of MTBC in smear-positive respiratory specimens.
Outpatient Order Accuracy
Authors: Valenstein Paul , Meier Frederick 
Published: January 2021

AbstractContext.—Laboratory test order entry errors potentially delay diagnosis, consume resources, and cause patient inconvenience.Objective.—To evaluate the frequency and causes of computer order entry errors in outpatients.Design.—Cross-sectional survey and prospective sample of errors. Participants answered questions about their test order entry policies and practices. They then examined a sample of outpatient requisitions and compared information on the requisition with information entered into the laboratory computer system. Order entry errors were divided into 4 types: tests ordered on the requisition, but not in the computer; tests performed but not ordered on the requisition; physician name discrepancies; and test priority errors.Participants.—Six hundred sixty laboratories enrolled in the College of American Pathologists Q-Probes program.Main Outcome Measure.—Overall order entry error rate.Results.—A total of 5514 (4.8%) of 114 934 outpatient requisitions were associated with at least 1 order entry error. The median participant reported 1 or more order errors on 6.0% of requisitions; 10% of institutions reported errors with at least 18% of requisitions. Of the 4 specific error types, physician name discrepancies had the highest error rate, and test priority errors the lowest error rate. Four institutional factors were significantly associated with higher overall error rates: orders verbally communicated to the laboratory; no policy requiring laboratory staff to compare a printout or display of ordered tests with the laboratory requisitions to confirm that orders had been entered correctly; failure to monitor the accuracy of outpatient order entry on a regular basis; and a higher percentage of occupied beds (ie, a busier hospital).Conclusions.—Computer order entry errors are common, involving 5% of outpatient requisitions. Laboratories may be able to decrease error rates by regularly monitoring the accuracy of order entry, substituting written and facsimile orders for verbal orders, and instituting a policy in which orders entered into computer systems are routinely rechecked against orders on requisitions.