bluuebunny/crossref_metadata_2025_split huggingface dataset:
Adenomatoid odontogenic tumor: a report of two unusually large lesions in the mandible
Authors: Lazim A , Kuklani R , Sundararajan D 
Published: October 2021

Abstract

Introduction/Objective
Adenomatoid odontogenic tumor (AOT) is an uncommon benign odontogenic tumor representing 3 to 7% of all odontogenic tumors. This tumor was first reported as adeno-ameloblastoma by Bernier and Tiecke in 1950 as it was initially assumed to be a type of ameloblastoma. In 1969, Philipsen and Bern proposed the term adenomatoid odontogenic tumor which was subsequently adopted by WHO and became the accepted terminology for this tumor. AOT is classified as a tumor of odontogenic epithelium but occasionally abnormal hard tissues consistent with dentinoid material may also be present as part of the tumor. AOT tends to occur in younger patients and 50% of the cases are diagnosed in teenagers. It occurs twice as commonly in females and frequently involves the anterior maxilla. Radiographically, AOT can appear as a radiolucent or mixed lucent-opaque lesion and may be associated with an impacted tooth.

Methods/Case Report
We report two unusually large expansile lesions of AOT that presented in the mandible. In the first case, the tumor presented as an expansile radiolucent lesion involving the right posterior mandible in a 32 year old female. In the second case, the tumor presented as an expansile mixed lucent-opaque lesion involving the left anterior mandible in a 21 year old female. The clinical presentation, radiographic and imaging findings, histopathologic features and treatment of these two cases will be discussed. The recommended treatment for AOT is surgical excision. The prognosis is good as this tumor seldom recurs after excision.

Results (if a Case Study enter NA)
NA

Conclusion
AOT is considered to be a non-aggressive, non-invasive and slow growing benign neoplasm. It is usually discovered on routine radiographic examination as the lesion is usually small and asymptomatic at the time of diagnosis but occasional cases that are larger in size have been reported in the literature.
Challenge in Early Diagnosis of Rare Lymphomas – A Case of Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Masquerading as Enteritis
Authors: Qiu W , Khokhar H , Dewenter T A , Jetly R 
Published: October 2021

Abstract

Introduction/Objective
Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an extranodal lymphoma arising from intestinal intraepithelial T-lymphocytes. Diagnosis is challenging obscured by non-specific gastrointestinal (GI) symptoms and radiographic findings, with potential delay in implementing therapy, as was encountered in a 40- year-old man of African-American descent with cachexia.

Methods/Case Report
The patient presented with a two month history of nausea, vomiting, diarrhea and 10-15 pounds weight loss. Computed tomography revealed wall thickening of multiple large- and small-bowel loops. The clinical differential diagnoses included infectious enteritis and inflammatory bowel disease. Since symptoms improved during the hospital stay, he was discharged with future outpatient endoscopy planned but returned a month later with abdominal swelling and pain. On repeat imaging, he had ascites and small-bowel mechanical obstruction versus ileus. Straw-colored ascites, dilated friable segments of small-bowel, matted mesenteric lymph nodes, and adhesions were found on exploratory laparotomy. Small-bowel segments and appendix were resected. A prolonged hospital course ensued post-operatively with death due to multiple complications.

Results (if a Case Study enter NA)
The intestinal wall was grossly thickened with no discrete masses and microscopically had extensive infiltrates of small- to intermediate-sized, mature, minimally pleomorphic, CD3+/CD4- /CD5-/CD7+/CD8+/CD10-/CD20-/CD30-/CD56+/EBER-ISH- T-lymphocytes, focally infiltrating into the epithelium. Ascitic fluid cytology had small lymphocytes, CD2+/CD3+/CD4-/CD5-/CD10-/CD7+/CD8+/CD25-/CD30-/CD56+ /TCRαβ+/TCRγδ- by flow cytometry immunophenotyping. A diagnosis of MEITL was rendered based on these findings.

Conclusion
MEITL is rare, aggressive, unassociated with celiac disease and usually seen in Asian and Hispanic populations. It presents with non-specific GI symptoms of abdominal pain, perforation, diarrhea, weight loss and intestinal obstruction underscoring the need for a high index of suspicion. Upper GI endoscopy has a limited role since involvement of small-bowel, specifically jejunum, is common. The evolving capsule endoscopy techniques may help in an early diagnosis. Due to limited literature, optimal treatment is unclear and various strategies using chemotherapy, surgery and stem-cell transplant have been tried with variable outcomes.
Immunohistochemical Characterization of H3K4Me3 in Reactive Lymph Nodes and Follicular Lymphoma
Authors: Xu G , Dresser K , Bledsoe J 
Published: October 2021

Abstract

Introduction/Objective
Genes involved in histone methylation are frequently mutated in non-Hodgkin’s lymphomas. For instance, frequent mutations in genes encoding histone methytransferases MML2 and EZH2 are present in diffuse large B cell lymphoma (DLBCL) and follicular lymphoma (FL). The aim of this study was to characterize the immunohistochemical expression of H3K4Me3 in benign/reactive lymph nodes (LNs) with comparison to follicular lymphoma (FL).

Methods/Case Report
Immunohistochemical staining with an anti-H3K4Me3 antibody was performed on FFPE whole slide section from patients with benign/reactive LNs (n=21), low grade (grade 1-2) FL (n=21). H3K4Me3 reactivity was scored for staining intensity and percentage of lymphocytes showing reactivity.

Results (if a Case Study enter NA)
The majority of the reactive LN sections (15 out of 21 cases) showed a distinct distribution of H3K4Me3 staining, with the majority of cells in the mantle zone and the interfollicular zones showing moderate-strong staining, whereas reactive germinal centers (GCs) showed significantly decreased or close to negative staining. Neoplastic follicles in all the FL cases contained positive cells with significantly stronger staining compared to that in the germinal centers in benign lymph nodes. The interfollicular zones, while diminished in FL due to expanded neoplastic follicles, showed retained H3K4Me3 staining. The difference in staining intensity between follicles and mantle/interfollicular zones became indistinct in FL.

Conclusion
H3K4Me3 expression in benign/reactive LNs is characterized by positive expression in lymphocytes in interfollicular and mantle zones and significantly decreased in GCs. However, the expression pattern is different in FL, which showed significantly increased expression in the follicles compared to that in reactive GCs. In FL, the expression in GCs is similar to that in the interfollicular and mantle zone lymphocytes. It is reported that about 90% of FL have MLL2 mutation and MLL2 is the enzyme responsible for the methylation of H3K4. So far, it is unclear whether the mutation of MLL2 in FL affects the overall methylation activity of the enzyme. In our study, there is increased methylation of H3K4 in the follicles of FL, which raises the possibility that MLL2 mutation somehow increases H3K4 methylation.
The Pathologic Spectrum of Bone Marrow Involvement by Double- and Triple-hit Lymphomas
Authors: Iqbal H , Harrington A , Kroft S H 
Published: October 2021

Abstract

Introduction/Objective
B-cell lymphomas with MYC and BCL2 and/or BCL6 rearrangements (double- and triple-hit lymphomas, DHL/THL) are a distinct entity due to shared biology and aggressive behavior, exhibiting poor outcomes with standard therapies. While pathologic features of DHL/THLs in primary sites have been well described, little information is available regarding the clinicopathologic features of bone marrow involvement by this entity.

Methods/Case Report
Files were searched from 2010-2020 for all DHL/THLs. Since mid-2016, all aggressive B-cell lymphomas were reflexed to DHL/THL FISH testing. Prior to that, criteria for performing FISH varied. Clinical and laboratory data were obtained through chart review. Both BM and primary diagnostic specimens were reviewed when possible.

Results (if a Case Study enter NA)
There were 46 DHL/THL cases with initial staging BM evaluations, of which 13 (28%) were positive for DHL/THL; 11 were available for review (5F:6M; 28-95 years). All patients with positive BMs were stage 3 or 4 irrespective of the BM findings. Lymphoma cytology in positive BMs was blastoid in 6, large cell in 2, and high grade, NOS in 3. The cytology in primary tissues was not significantly associated with the rate of marrow involvement. PB smears were available for 9/11 BM(+) cases; of these, 6 (66.7%) had circulating lymphoma cells in the blood, ranging from rare to greater than 40% lymphoma cells (median, 4%). Lymphoma cells with cytoplasmic vacuoles were present in 5 cases (45%). No BM infiltrates had a starry-sky appearance. Infiltration patterns included diffuse (3), diffuse and interstitial (3), and interstitial (3). One exhibited only rare, scattered lymphoma cells in the aspirate and core biopsy, and another with large cell morphology showed random focal (nodular) and focally paratrabecular infiltration. The proliferation index in the marrow infiltrates ranged from 50% to &gt;90% (median, 65%). Flow cytometry was positive in 9 of 10 cases; the single negative study was from an outside institution

Conclusion
Our study demonstrates 28% of DHL/THLs show BM involvement at diagnosis. Notably, the peripheral blood was involved in 2/3 of cases with marrow infiltration (13% of total cases), ranging from rare circulating cells to frank leukemic involvement. Cytologically, the marrow infiltrates were predominantly blastoid or high grade NOS. Marrow infiltrates generally displayed leukemic rather than lymphomatous patterns of involvement.
PAX5 P80R mutated acute leukemia followed by genetically-related histiocytic proliferation with clonal IGH gene rearrangements
Authors: Wu A , Cheng L , Vance G H , Zhou J , Gulati R , Dave U , Czader M 
Published: October 2021

Abstract

Introduction/Objective
PAX5 is a transcription factor critical for B-cell development and its genetic alterations are common in B lymphoblastic leukemia/lymphoma (B-ALL). We report a case of PAX5 P80R mutated acute leukemia with predominantly monocytic immunophenotype followed by a genetically-related histiocytic proliferation.

Methods/Case Report
A 27-year-old male presented with pancytopenia, epistaxis, and blurry vision. Bone marrow exam showed 95% blasts with nuclear indentations, occasional prominent nucleoli and basophilic cytoplasm. Blasts were positive for bright CD33, HLA-DR, CD14, bright CD64, partial CD123 and partial CD19. In addition, a minute population of B lymphoblasts positive for CD19, CD20, and dim TdT was seen. The AML FISH panel showed markedly aneuploid population with all probes demonstrating 3 to 5 signals. PAX5 P80R and KRAS G13D mutations were detected by NGS. Post induction bone marrow showed no evidence of acute leukemia with normal cytogenetics and FISH results. Subsequent two bone marrow exams performed due to progressive cytopenias demonstrated a prominent histiocytic proliferation with sheets of mature appearing histiocytes with abundant cytoplasm, oval to folded nuclei and occasional hemophagocytosis. This population was positive for CD163, CD14, CD68R, CD68, cyclin D1 with variable OCT2 expression and low proliferative activity. PAX5 P80R and KRAS G13D were detected. AML FISH panel showed aneuploidy in histiocytic appearing cells and IgH gene rearrangement studies by PCR showed prominent clonal population. The patient remained pancytopenic and died of disseminated fungal infection.

Results (if a Case Study enter NA)
NA

Conclusion
PAX5 P80R mutation has been primarily reported in B-ALL and is exceedingly rare in acute myeloid leukemias. This alteration has been linked to downregulation of B-cell lineage genes affecting B-cell maturation, and not surprisingly a proportion of PAX5 P80R mutated B-ALL cases show a switch to a monocytic lineage. The reported case demonstrates diagnostic caveats including unusual features of acute leukemia at the time of initial diagnosis and subsequent genetically-related histiocytic proliferation.
Blinatumomab resistant clone presenting as mixed phenotype myeloid sarcoma causing cord compression
Authors: Dabrowski D S , Wei E 
Published: October 2021

Abstract

Introduction/Objective
Blinatumomab is a monoclonal antibody directed against CD19/CD3 utilized for the treatment of relapsed or refractory B-cell precursor acute lymphoblastic leukemia (ALL) and for the treatment of B-cell precursor ALL in first or second complete remission with minimal residual disease (MRD) ≥0.1%. Although Blinatumomab treatment has shown better overall survival, progression-free survival, and complete remission when compared to chemotherapy, most patients have a relapse and ultimately succumb to the disease. Interestingly, there are a number of cases reporting relapse in extramedullary places. The mechanisms for relapse in these unusual extramedullary sites are not well-understood. We herein report a case of a 20-year-old African American male with primary refractory Philadelphia-negative (Ph-) precursor B cell ALL with MLL rearrangement, who received treatment with Blinatumomab after achieving morphological remission with a pediatric-inspired regimen but found to be MRD +.

Methods/Case Report
A 20 year old African American male was found to have B cell precursor ALL. It was found to be Ph-. While initally receiving vincristine, prednisone, and aspariginase, the ALL proved to be refractory to treatment. Blinatumomab was used as second line therapy after the first failed remission. The patient remained with morphological response; however, remained MRD+ after three cycles of Blinatumomab. During the fourth cycle, the patient presented with back pain and lower extremity weakness. A spine MRI revealed an extradural mass in the thoracic spine causing cord compression. A thoracic laminectomy with partial removal of the mass, followed by radiation, was performed with improvement of symptoms. Pathology results of the extradural mass revealed a myeloid sarcoma with MLL rearrangement.

Results (if a Case Study enter NA)
NA

Conclusion
This case report demonstrates how patients treated with blinatumomab can have relapse in unusual extramedullary places. The possibility of leukemia manifesting in extramedullary sites should always be kept in mind by clinicians treating patients with Blinatumomab. The mechanisms of resistance against Blinatumomab are not well- understood and need further elucidation.
Kinetics of Eosin-5-maleimide Binding to Red Blood Cells
Authors: Ventura M 
Published: October 2021

Abstract

Introduction/Objective
The binding of eosin-5-maleimide (EMA) to red blood cells in different pH conditions has not been well-investigated. The current standard protocol uses phosphate buffered saline at pH 7.0 to 7.5 for the binding reaction. The reaction typically uses a sixty-minute incubation. However, the effect of pH on EMA binding remains to be determined, nor have the reaction times at different pH conditions. This study utilizes optimal pH conditions of 6.0, 6.5, 7.0, 7.5, 8.0, and 8.5 to characterize EMA binding. The two parameters, time, and pH were investigated to extensively describe the binding kinetics of eosin-5-maleimide.

Methods/Case Report
The working EMA solutions in phosphate buffered saline at different pHs (specifically 6.0, 6.5, 7.0, 7.5, 8.0, and 8.5) were incubated in the dark with 1 µL of washed red blood cells and 25 µL of working EMA solution. The red blood cells and working EMA solution were vigorously mixed before the specified incubation time. The binding was recorded at specified time points, namely 1, 2, 4, 8, 16, 32, and 64 minutes for each pH condition. The binding reaction was terminated by washing three times with 0.1% bovine serum albumin (BSA) in PBS and centrifuged at 400 g for 5 minutes. The red blood cells were then resuspended with 500 µL of PBS and ran in the BD FACSLyric™ flow cytometer.

Results (if a Case Study enter NA)
The current EMA binding protocol utilizes a. neutral pH of 7.0 to 7.5 recorded at 64 min. Based on our data, better discrimination of hereditary spherocytosis red blood cells from normal red blood cells may be obtained at a more basic pH of 8.5. Thus, a statistical t-test was performed for the comparison between the current setup and the apparent optimal pH conditions at shorter reaction times of 16 min and 32min at pH 8.5.

Conclusion
The resulting data suggest that enhanced discrimination of HS red blood cells can be achieved in a more basic pH. This was evidently observed at pH 8.5, where the highest percentage decrease is recorded, and the most significant difference compared to the other pH conditions. In conclusion, the study identifies that better discrimination of hereditary spherocytosis red blood cells from normal red blood cells can be determined using pH 8.5 with a shorter incubation time of 32 min. The pH 8.5 at 16 min condition could also be considered if the clinical batch size allows precise incubation of 15 min.
Concordance and discordance among antiphospholipid syndrome screening assay
Authors: Reno M , Stefaniuk C 
Published: October 2021

Abstract

Introduction/Objective
The lupus anticoagulant (LA) panel utilizing dilute Russell’s viper venom and LA-responsive APTT remains the front-line screen for detecting the presence of clinically significant antibodies in the diagnosis of antiphospholipid syndrome. Receiving anticoagulant therapy limits the utility of clotting-based methods due to increased risk of false positive results. Anti-cardiolipin (aCL) and anti-ß2-glycoprotein I (a2GßPI) antibody titers have also been employed for the detection of anti-phospholipid antibodies; however, they are not influenced by anticoagulation therapy. The International Society on Thrombosis and Haemostasis recommends that all three tests should be performed to appropriately assess the risk of lupus anticoagulant/antiphospholipid antibodies. In this study, the concordance and discordance of rates of among these three assays were examined.

Methods/Case Report
The electronic medical record was mined according to lab tests codes for the results of the LA panel, aCL and a2GßPI antibody titers for all patients having at least one resulted study at the University of Cincinnati Medical Center in Cincinnati, Ohio, over a one-year period (2020). Results were compared to determine rates of concordance and discordance among the assays as well as Spearman’s correlation. Non-parametric binary regression analysis was applied to explore the relationship connecting aCL and a2GßPI titers to LA panel findings.

Results (if a Case Study enter NA)
Strong concordance was seen between the results of LA panels and antibody titer assays. A weak but significant positive correlation was found among the three assays. A robust logarithmic relationship was developed from which the LA panel result could be predicted utilizing outcomes from aCL and a2GßPI antibody titer findings.

Conclusion
These data further support the reliability of aCL and a2GßPI titers in the evaluation of antiphospholipid syndrome. Furthermore, the robust relationship among these three assays suggests that antibody titers may provide a reliable diagnostic avenue for patients actively undergoing anticoagulation therapy.
Individual and Combined Evaluation of Specific Inflammatory Biomarkers along with Neutrophil, Lymphocyte, and Platelet Counts in Predicting Poststroke Disability Severity Among Acute Ischemic Stroke Patients in a Tertiary Hospital in Pampanga, Philippines
Authors: Marcadejas E R , Lagman M P , Mendoza A S , Marzan R E , Manongdo A Z , Maniego L S , Meriales M M , Marin E P , Sison R G , Nieto X A 
Published: October 2021

Abstract

Introduction/Objective
Numerous studies considered Neutrophil-to-Lymphocyte ratio (NLR), Platelet-to-Lymphocyte ratio (PLR), and Mean Platelet Volume (MPV) as inflammatory biomarkers in assessing the functional outcomes of Acute Ischemic Stroke (AIS) Patients. Presumably, no other study has evaluated all three markers in varying levels of combinations. The study aims to evaluate the predictive potential of specific CBC parameters (Neutrophils, Lymphocytes, and Platelets), their individual ratios (NLR, PLR, and MPV), and their combinations (NLR-PLR, NLR- MPV, PLR-MPV, and PLR-NLR-MPV).

Methods/Case Report
This retrospective study involved 52 AIS patients from a hospital in Pampanga, Philippines, at least 18 years of age, have no pre-existing conditions and prior treatments/medications. Modified Rankin Scale (mRS) score, medical history, age, and gender were gathered. Specific CBC parameters upon admission were analyzed to derive individual ratios. The combined biomarkers, categorized into Levels 2, 1, and 0, indicate high values for all biomarkers involved, high values for any of the biomarkers (one or two high value/s for PLR-NLR-MPV), and no high values for any of the biomarkers, respectively.

Results (if a Case Study enter NA)
Results showed that elevated NLR (OR=14.5; p=0.021) and MPV (OR=24.1; p=0.047) are risk factors in developing evident poststroke disability (mRS 2-5) and mortality (mRS 6), respectively. Furthermore, level 2 NLR-MPV (OR=77.0; p=0.040) and PLR-MPV (OR=105; p=0.027) are risk factors on mortality. Level 2 PLR-NLR (OR=15.0; p=0.021) and level 1 NLR-MPV (OR=13.5; p=0.024) are risk factors in developing evident poststroke disability. Lastly, levels 1 (OR=13.5; p=0.024) and 2 (OR=77.0; p=0.040) PLR-NLR-MPV are risk factors in developing evident poststroke disability and mortality, respectively.

Conclusion
Patients with neutrophilia, elevated NLR, level 2 PLR-NLR, and level 1 NLR-MPV and PLR-NLR-MPV are more likely to develop evident poststroke disability; while patients with elevated MPV and Level 2 NLR-MPV, PLR- MPV, and PLR-NLR-MPV pose higher mortality risk. Greater sample size is recommended for studies with the same purpose to advance research for better AIS outcomes.