bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cystic Fibrosis–Related Liver Disease in a 12-Year-Old: Important Questions of Portal Hypertension Etiology That Start With Pathology: A Case Presentation
Authors: Burkholder Ryan , Ozluk Ekin , Cruz Nestor E Dula 
Published: September 2019

Abstract

Introduction
Cystic fibrosis liver disease (CFLD) has long been postulated to be secondary to dysfunctional cystic fibrosis transmembrane conductance regulator in the apical biliary epithelium, leading to bile stasis and eventually cirrhosis with portal hypertension (PH). We present a considerably young patient with signs of esophageal varices and liver biopsy indicating CFLD with significant obliterative portal venopathy as a result of long-standing portal hypertension.

Case Presentation
A 12-year-old with CF (diagnosed at 1 year) and pancreatic insufficiency was noted to have chronically elevated liver enzymes with decreasing platelet counts (130,000 K/ µL). These findings prompted an ultrasound, which showed splenomegaly. Upper endoscopy showed grade 1 esophageal varices, and liver needle biopsy performed at this time demonstrated focal neutrophilic lobular inflammation with marked pseudoacinar transformation and biliary metaplasia. Noted was grade 1 macrovesicular steatosis, focal dense fibrosis also known as “focal biliary cirrhosis,” and obliterative portal venopathy.

Discussion
CF patients with liver fibrosis have significant risk of future morbidity. Of specific interest is identification of PH, and obliterative portal venopathy is a very important feature to distinguish on biopsy as it predicts clinical course. Two recent studies in young adults described obliterative portal venopathy (OPV) and noncirrhotic portal hypertension (NCPH) as the predominant pathophysiology in young adults (median, 22 years) with CFLD. One study found cirrhosis present in only 27% of patients with portal hypertension. In addition, it appears that portal hypertension can precede cirrhotic changes. This unique case in a very young patient with CFLD demonstrates that the underlying pathogenesis of noncirrhotic portal hypertension and CFLD remains to be fully solved. These observations may have important consequences for CFLD management, including opting away from “cholestasis-targeted” treatments and more often considering portosystemic shunting procedures instead of transplant, as liver function remains preserved in numerous CFLD cases.
An Interesting Case of EBV-Associated Autoimmune Hemolytic Anemia
Authors: Desai Sapna , West Jeffrey , Sena Salvador 
Published: September 2019

Abstract
We report a case of a 28-year-old male with no significant past medical history who presented with a weeklong history of flu-like symptoms, dark urine, and cold-induced pain and discoloration in his fingers. Upon presentation, he was noted to be significantly jaundiced. Abdominal ultrasound showed splenomegaly. Laboratory test results returned as follows: hematocrit, 26.4%; hemoglobin, 9.0 g/dL; WBC count, 9.8 × 109/L; and automated differential, 0.42 segmented neutrophils, 0.44 lymphocytes, and 0.08 monocytes. Manual differential showed 0.26 atypical lymphocytes, 0.21 lymphocytes, 0.10 monocytes, 0.03 metamyelocytes, and 0.05 segmented neutrophils. He was found to have a normochromic macrocytic anemia with absolute lymphocytosis, monocytosis, and thrombocytopenia. Peripheral blood smear revealed normochromic macrocytic RBCs with anisocytosis. Downey type II cells, plasmacytoid lymphocytes, large activated lymphocytes, and monocytes were present in addition to a neutrophilic left shift up to metamyelocytes. Liver function tests showed elevated levels of total bilirubin (8.4 mg/dL), direct bilirubin (2.6 mg/dL), aspartate aminotransferase (193 U/L), alanine aminotransferase (102 U/L), and alkaline phosphatase (132 U/L). Urinalysis was positive for urobilinogen. Serology was nonreactive for anti–hepatitis A IgM, hepatitis B surface antigen, and anti–hepatitis C antibody. Positive results were obtained for a rapid monoscreening test and Epstein-Barr virus viral capsid antigen IgM. Direct antiglobulin testing showed positivity for IgG, and complement and cold autoantibody were detected. He was transfused and treated with prednisone, IVIG, and external warmth. After improvement, the patient was discharged with recommendations for follow-up. Hemolytic anemia is a relatively rare complication of patients with infectious mononucleosis and occurs in approximately 1% to 3% of these patients. The pathogenesis of EBV-related autoimmune hemolytic anemia is unknown, with a suspicion for inadequate B-cell function after infection. Appropriate laboratory testing and analysis are critical for rapid diagnosis in these patients to ensure adequate treatment with no long-term sequelae.
CSF Autoantibody Panel Test Utilization in Patients Presenting With Neurologic Alterations: A 3-Year Retrospective Study
Authors: Salazar Cristo Guardado , Carayannopoulos Mary 
Published: September 2019

Abstract

Introduction
When patients present with a range of neurological symptoms, it can be difficult to identify the cause. CSF evaluation of patients who present with a subacute neurological disorder of undetermined etiology is very helpful, especially in patients with known risk factors for autoimmune neurologic disease or cancer. The goal of this study was to review the results of CSF autoantibody panels ordered over the last 3 years and determine the percentage of panels that were positive.

Methods
Results of CSF autoantibody panels ordered over the last 3 years were reviewed and the percentage of samples that tested positive and the most common autoantibody was calculated.

Conclusion
At our institution, pathology residents review orders for CSF autoantibody panel evaluation and approve testing based on the clinical history and neurologic evaluation in order to prevent inappropriate test utilization. Of the 192 CSF evaluations ordered between 2015 and 2018 (5 dementia, 72 encephalopathy, and 115 paraneoplastic CSF-autoimmune evaluation), 19.8% of the tests were positive. The most common autoantibodies detected were the NMDA (4.68%) and VGKC (4.68%) receptors.
Concordance Between the TIRADS Ultrasound Scoring Criteria, Fine-Needle Aspiration Cytology, and Thyroid Final Resection Diagnosis
Authors: Kaseb Hatem , Charifa Ahmad , Abi-Raad Rita , Cai Guoping , Hammers Lynwood , Prasad Manju , Adeniran Adebowale 
Published: September 2019

Abstract

Objectives
Thyroid imaging reporting and data system (TIRADS) criteria were recently introduced in our institution to aid in predicting diagnosis for various thyroid lesions. We evaluated the association of TIRADS imaging score and fine-needle aspiration (FNA) cytology with thyroid lesions that had a confirmed diagnosis at resection, with a focus on understanding the predictability of this diagnostic tool in malignancy prediction.

Methods
We assessed the concordance of TIRADS criteria and FNA diagnosis to the final anatomical diagnosis in the assessment of thyroid lesions. We retrieved the cases from the archives of the Yale pathology department between June 2017 and January 2018. Our inclusion criteria included patients who had a TIRADS score, cytology diagnosis, and final surgical resection diagnosis. A total of 65 subjects with an age range of 11 to 88 years were identified.

Results
The majority of the patients were females, 65% (42/65). Cases with TIRADS score 1-2 (likely benign) and Bethesda I/II nondiagnostic/benign were few since most of these cases did not go for surgical resection. The mildly suspicious TIRAD score 3 and FLUS category showed similar trends, 68% and 67%, respectively, in predicting malignant lesions. The TIRADS score 4 when compared to cytology (IV)/(V) demonstrated similar consistent results in malignancy prediction, both being high at 89% and 87%, respectively. The TIRADS score 5 demonstrated a 95% malignancy prediction. The overall sensitivity and specificity of TIRADS score in our cohort were 66% and 77%, respectively. The positive and negative predictive values of TIRADS score in our cohort were 89% and 39%, respectively. In comparison, the overall sensitivity and specificity of cytology assessment in our cohort were 91% and 44%, respectively. The positive and negative predictive values of cytology assessment in our cohort were 85% and 57%, respectively.

Conclusion
Our results demonstrated that both cytology and TIRAD score had similar trends in malignancy prediction. Cytological assessment had higher sensitivity but lower specificity compared to TIRADS score. While both techniques showed concordant high predictability of malignant lesions (approximately 91%), the use of both modalities adjunctively will be very useful in triaging cases for surgery. Overall, utilizing TIRADS score with cytology will help reduce the risk of unnecessary invasive procedures in patients with a low probability of malignant thyroid disease.
PD-L1 Expression on Cell Block Preparations of Pancreatic Adenocarcinoma
Authors: Costa Victoria , Kim David , Johncilla Melanie , Goyal Abha , Rao Rema 
Published: September 2019

Abstract

Objectives
Programmed death-ligand 1 (PD-L1) is an emerging molecular target in anticancer therapy, most notably non–small cell lung cancers. PD-L1 expression in pancreatic adenocarcinomas (PDAs) on surgical specimens is highly variable, with 10% to 60% of tumors showing expression. PD-L1 expression in PDA on endoscopic ultrasound-guided fine needle aspirations (EUS-FNAs) has been rarely studied.

Methods
Formalin-fixed, paraffin embedded (FFPE) cell blocks (CBs) from 65 EUS-FNA samples of 55 patients, with a diagnosis of PDA, with at least 20% tumor cellularity were retrieved. The cell blocks were originally fixed in CytoRich fixative. Immunohistochemical staining (IHC) for PD-L1 was performed using the M365329-1 (22C3) clone according to the manufacturer’s approved protocol and optimized for the fixation method using appropriate controls. A combined positive score (CPS) defined per CAP guidelines as the total number of positive tumor cells and inflammatory cells as a percentage of the total number of tumor cells was assessed for each case and was grouped as <1, 1-20, or >20.

Results
Twenty-five samples (38%) from 21 patients showed immunoreactivity to PD-L1, with 21 (32%) having a CPS of <1 and four (6%) having a CPS of 1-20. Eight of these 25 samples had surgical correlates, of which concordant staining was noted in five (62.5%). Of the discordant three, decreased tumor cell sampling in the core biopsy was noted in one. Overall, 20 of 21 patients (95%) with PD-L1 immunoreactivity had disease progression with 17 (81%) associated with metastatic disease and three (14%) with locally advanced disease.

Conclusion
Immunohistochemical analysis for PD-L1 is feasible on EUS-FNA CB samples when optimized and validated for the fixation method using appropriate controls. PD-L1 expression is seen in only a minority of PDAs, albeit with a very low CPS. The potential role of PD-L1 as a prognostic marker for disease progression needs further exploration.
Where in the World Is . . . My Cytology Specimen? Using Lean Six Sigma Methodology in Specimen Management
Authors: Cardenas Deb , Walls Michelle , Williams Michelle , Gehman Mary , Armentrout Alvin , Sudor Janine , Stipe Thomas , Woodbury Sara 
Published: September 2019

Abstract

Introduction
An automatic signal was not in place to actively identify when a cytology specimen resulted more than 28 days from collection. The project focus was to decrease the number of specimens resulted more than 28 days and improve result cycle time to enhance safe, quality care delivery.

Methods
Annually, more than 46% of the cytology specimens processed at our academic medical center originate from our Women’s Health practice site. A Lean Six Sigma Green Belt project commenced with key stakeholders from the practice site, Cytology and Clinical Laboratory Departments. The project goal was to reduce the percentage of specimen results taking longer than 28 days from the baseline of 6.7% to ≤1.7%. Voice of the customer showed 75% of patients would be satisfied with results available within 7 days. Baseline data showed 38.6% of specimens resulted within 7 days with an improvement goal of 85%.
Lean Six Sigma methodology was employed to develop standard work. The Cytology Department implemented use of work lists in the CoPathPlus system to track specimens from accession to results. Reconciliation of cytology specimens received versus ordered was performed weekly.

Conclusions
Baseline data were compared to postprocess implementation data. Hypothesis testing compared baseline to postimplementation cycle time means showing a reduction from 11.28 to 5.15 days (P < .001). Project goal of ≤1.7% for results greater than 28 days was exceeded at 0.22%. Cycle time goal of 85% for results within 7 days was exceeded at 91.6%.
Signal presence to identify results greater than 28 days was reduced with improved cycle time for results reporting. With standard work implementation, 616 annualized labor hours and an annualized labor cost reduction of more than $19,000 resulted. The standard work developed has the capability to translate across all practice locations where cytology specimens are obtained.
Solid Variant of Serous Cystadenoma of the Pancreas, Cytology Findings
Authors: Nowroozizadeh Behdokht , Vanleer Jessica , Goreal Wamda 
Published: September 2019

Abstract

Introduction
Serous cystadenoma of the pancreas is the most common benign neoplasm of the pancreas, tending to have a very good prognosis and treatable by surgical excision. The solid variant of serous cystadenoma of the pancreas is a very rare subtype with only a few cases reported in recent literature, mostly in elderly females.

Case History
An asymptomatic 85-year-old woman presented with a large pancreatic mass. Endoscopic ultrasound findings were consistent with an ill-defined large isoechoic mass involving almost the entire pancreas, with no significant common bile duct dilation. Fine-needle aspiration was performed.

Results
The smears showed groups of cells with uniform nuclei, ample cytoplasm, inconspicuous nucleoli, minimal cytologic atypia, and absent mitotic activity, suggesting a benign or low-grade lesion. A solid aggregate of these cells was present in the cell block, which was positive for CK7, inhibin, and PAS. Negative staining for chromogranin, synaptophysin, and B-catenin helped exclude neuroendocrine tumors and solid pseudopapillary neoplasm. Additionally, absent staining for PAX-8, ER, PR, BRST-2, GATA-3, CK20, CDX2, Melan-A, and calretinin helped exclude other metastatic clear cell lesions.

Conclusion
Diagnosis of solid variant serous cystadenoma of the pancreas can be challenging due to nonspecific clinical symptoms and misdiagnosis as other solid pancreatic tumors. Based on the clinical, radiologic, cytologic, and immunohistochemical features, we favor a diagnosis of solid variant of serous cystadenoma. Recognition of this lesion is important because the vast majority of solid tumors in the pancreas are malignant. The differential diagnosis includes the rare primary clear-cell “sugar” tumor of the pancreas, clear cell carcinoma, clear cell islet cell tumor, and metastatic renal cell carcinoma. Therefore, the diagnosis of solid-type serous cystadenoma of the pancreas is frequently dependent on a series of immunohistochemical and molecular studies.
Adequacy and Incidence of Primary Pancreatic Cancer on Endoscopic Ultrasound-Guided Fine-Needle Aspiration (EUS-FNA) Cytology Samples: A Retrospective Review in Veteran Population at Corporal Michael J. Crescenz Veteran Affairs Medical Center (CMCVAMC)
Authors: Dalal Sharvari , Petersen Jeffrey , Jhala Darshana 
Published: September 2019

Abstract

Introduction
Pancreatic cancer is the one of leading causes of cancer-related deaths, accounts for 7% of cancer related deaths, and generally is diagnosed at an advanced stage. The 5-year survival rate is about 62%. There are no current screening recommendations for pancreatic cancer, so primary diagnosis is of the utmost importance. EUS modality for FNA has revolutionized the ability for the early diagnosis and staging of cancers of the gastrointestinal tract as being safe, cost-effective, minimally invasive technology to assess the pancreas. To date, there is no study in English literature assessing the adequacy for diagnosis and ancillary studies on EUS-FNA of pancreatic mass lesions in veterans. The veteran patient population is a unique cohort with different risk factors and demographics.

Methods
As a part of quality assurance project, a search was conducted to identify all EUS of pancreatic lesions performed at our hospital from February 2, 2011, to March 3, 2019. We evaluated adequacy on the cell blocks and diagnostic accuracy of EUS-FNA among all pancreatic mass lesions.

Results
Out of the total 130 EUS-FNA pancreas cases, the diagnosis was established in 127 cases (97.6%). Three cases were insufficient and total 26 cases were diagnosed as atypia, suggesting repeat FNA for definitive diagnosis. A definitive diagnosis was made in 101 cases (77.7%). Four cases were suspicious for malignancy. Malignancy/severe dysplasia was diagnosed in total 45 cases, most being primary pancreatic adenocarcinoma (41) and four with other diagnoses.

Conclusion
EUS-FNA is highly successful for procuring adequate material on the cytology samples for pancreatic lesions. EUS-FNAs for pancreatic lesions have high sensitivity and specificity for diagnosing and directing early, appropriate clinical management in veterans. The percentage of primary pancreatic adenocarcinomas out of total tumors in this veteran population is high (91.1%).
Cytologic Features of Lymphoepithelioma-Like Carcinoma Involving the Parotid: A Case Report
Authors: Chen Fei , Modi Lopa , Zamuco Ronaldo , Yee-Chang Melissa 
Published: September 2019

Abstract
Fine-needle aspiration biopsies (FNABs) are a common modality used in the evaluation of salivary gland neoplasms. We present the cytologic and histologic features of a rare case of lymphoepithelioma-like carcinoma (LELC) in a 40-year-old Hispanic male with a 1.0-cm painless well-circumscribed parotid mass that had been present for 8 years. FNAB smears showed cohesive groups of intermediate-sized basaloid cells with vesicular nuclei, occasional pleomorphic nuclei and prominent nucleoli, and spindled morphology. Mature lymphocytes were seen in the background, either adjacent to the atypical epithelial cells or dispersed in the background. This lymphoid background raises considerations of salivary gland neoplasms that can have prominent lymphocytic backgrounds, such as acinic cell carcinoma and, more commonly, Warthin’s tumor or metastasis involving intraparotid lymph node. Surgical resection of the parotid showed syncytial sheets of predominantly undifferentiated cells with spindled to epithelioid morphology and occasional prominent nucleoli and focal areas of squamous differentiation. The background showed dense areas of lymphocytes with germinal center formation. Immunohistochemical (IHC) stains showed positive reactivity for p63, p40, and EBV in situ hybridization (EBV ISH) in the tumor cells and negative reactivity for p16. The findings were supportive of LELC if a metastasis from the nasopharynx was excluded. A subsequent nasopharyngeal biopsy was benign. Although histologic features of LELC are well established, we identified rare case reports describing the cytomorphology in the literature. Cytopathologists should be aware of this lesion as another salivary gland neoplasm that can show lymphocytes admixed with the tumor cells and a distinct lymphoid background. The basaloid appearance and cytologic atypia should distinguish it from acinic cell carcinoma and Warthin’s tumor. However, metastatic lesions should also be considered with a distinct lymphoid background and need to be clinically excluded before establishing the diagnosis of LELC.
Training of Super Users Contributes to Productivity in Molecular Biology Laboratories in Mozambique
Authors: Assane Mariamo , Basu Debby , Landgraft Kenneth , Pinto Isabel , Adamo Noorbebi , Macave Admiro , Nhabomba Augusto , Benzane Octavia 
Published: September 2019

Abstract
HIV viral suppression through antiretroviral (ARV) treatment has public health benefits in potentially reducing the risk of subsequent HIV transmissions. Mozambique has adopted the World Health Organization (WHO) “Test and Start” strategy, which assumes that all people who test positive for HIV start ARV treatment immediately. In order that treatment is guided by the respective HIV viral load (VL) test results, the MOH expanded the network of VL testing laboratories. About 26 VL testing instruments are now operational in the country. Although the increase in testing platforms increased VL tests performed, problems associated with errors, failures in user maintenance, and equipment malfunctions occur frequently. Delays in resolving equipment malfunctions contributed to lower laboratory productivity in certain periods of 2018. Therefore, the MOH, in coordination with the American Society for Clinical Pathology (ASCP) and VL testing instrument manufacturers, provided targeted training for VL laboratory Super Users (SUs). The SUs are primarily composed of laboratory technicians representing all VL laboratories. Training focused on equipment maintenance, software configuration, and troubleshooting the most common instrument-related problems and errors. Following this training, the SUs worked as in-laboratory equipment supervisors, helping laboratory staff to better perform equipment maintenance procedures. Furthermore, they worked remotely with representatives and field technicians of the instrument manufacturers to resolve small problems, such as accessory replacement and adding new users to the systems, in their own laboratories. This has led to a reduction in the overall time to equipment repair, less reliance on external field technicians, and correspondingly increased overall lab productivity and workforce capacity building for VL laboratory SU.