bluuebunny/crossref_metadata_2025_split huggingface dataset:
Gluteal Region Plasmablastic Lymphoma in a Crohn’s Disease Patient Treated with Anti-Tumor Necrosis Factor and Immunosuppressive Therapies: Report of a Rare Case with Literature Review
Authors: Sadough Shahmirzadi M , Fakhri N , Jetly R , Loganantharaj N 
Published: November 2022

Abstract

Introduction/Objective
Plasmablastic lymphoma (PBL) is rare, initially described in the oral cavity of HIV patients but can affect other extra-nodal sites and immunodeficiency states. We report an unusual case of PBL in an HIV negative Crohn’s disease (CD) patient with prior anti-tumor necrosis factor (TNF) and immunosuppressive therapies including prednisone.

Methods/Case Report
A 54-year-old man with recent bleeding from chronic gluteal wounds had CD (five years), a left-lower-quadrant diverting colostomy (three years) and treatment with adalimumab (three years), briefly with infliximab (discontinued after an infusion reaction) and chronic prednisone. Multiple perianal fistulous tracts were present and biopsied. Dermal diffuse infiltrate of pleomorphic plasmacytoid cells had prominent nucleoli, and an immunohistochemical profile as: CD20-/CD22-/CD30-/CD38-/CD45-/CD56+(weak, subset)/CD79a-/CD138+/EBV+(EBER-ISH)/HHV8-/kappa+/lambda-/MUM1+/PAX5-/Ki-67(70-80%) confirming PBL. Chemotherapy with bortezomib combined with etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin resulted in a good response.

Results (if a Case Study enter NA)
NA.

Conclusion
A meta-analysis found increased risk of non-Hodgkin lymphomas in CD treated with anti-TNFs, however, lymphoma subtypes were not specified. Seven cases of CD-associated PBL were found in the literature (from 2007). Of all cases 6 were men and 2 women with an average age 49 years (range 32-65), HIV negativity documented in five, involvement sites of gastrointestinal tract (5), perianal skin (2) and paravertebral soft tissue (1), and treatments with anti-TNFs (6), 6-mercaptopurine+budesonide (1) and salazosulfapyridine (1). The average intervals from CD and anti-TNFs to PBL were 14.5 years (range 5-30) and 17 months (range 4-36) respectively. Increased PBL risk may be related to the disease itself, anti-TNF/immunomodulator therapies, EBV reactivation, or other host-related factors. Investigations for potential risks of CD therapies can ensure their safe use in susceptible patients.
Alpha Heavy Chain Disease Post-Diffuse Large B-cell Lymphoma Remission: A Series of Two Cases
Authors: Aljumaili Z , Ahmed A , mai B , Wahed M 
Published: November 2022

Abstract

Introduction/Objective
Heavy chain disease is the production of abnormal monoclonal immunoglobulin heavy chains. Alpha heavy chain disease is typically identified in mucosa-associated lymphoid tissue lymphomas and is secreted due to chronic infection. The alpha heavy chain can manifest as hypogammaglobulinemia or a normal serum protein electrophoresis (SPEP). Broadband may appear in the alpha-2 or beta mobility regions.

Methods/Case Report
We describe two cases in which a monoclonal alpha heavy chain was detected in a 60-year-old female with diffuse large B-cell lymphoma (DLBCL) of the thoracic spine and a 71-year-old female with mediastinal DLBCL. Both patients achieved clinical and radiological remission.

Results (if a Case Study enter NA)
A distinct IgA band without an associated light chain was detected on the first patient's serum immunofixation essay (SIFE) one year later. For the second case, there was a distortion of the beta-2 region, elevated kappa free light chain (230.6 mg/dL), and a kappa to lambda free light chain ratio of 14.23, suggesting alpha heavy chain disease and a possible monoclonal gammopathy of unknown significance. Interestingly, even with a monoclonal IgA, the patients remained symptom-free, without any gastrointestinal manifestations, and concurrent PET scans show no recurrence.

Conclusion
To the best of our knowledge, these are the first two cases describing the presence of alpha heavy chain disease in association with DLBCL. The significance of alpha heavy chain secretion in patients with a history of DLBCL needs to be further assessed and we need to identify the possible presence of a non-clonally related paraprotein post-treatment for DLBCL.
High-Grade B-Cell Lymphoma with Effusion as the Initial presentation
Authors: Aljumaili Z , mai B , Ahmed A , Wang X , Zhang H , Hu Z 
Published: November 2022

Abstract

Introduction/Objective
Malignant effusion is usually caused by metastatic carcinoma. Lymphoma is often not included in the differential diagnoses. Here, we describe two cases of young females with no significant past medical history, presenting with fluid overload.

Methods/Case Report
The first case is a 41-year-old female with abdominal ascites and pleural effusion. Microscopic examination of the ascitic fluid showed intermediate to large lymphoid cells with irregular nuclear contours and fine chromatin. Flow cytometry showed CD10-positive lambda restricted B-cells. She had retropectoral mass biopsy showing sheets of intermediate to large-sized lymphoma cells with abundant apoptotic bodies and necrosis. The lymphoma cells are positive for CD20, CD10, BCL-2, BCL-6, and C-MYC (~50%,) while negative for TdT and cyclin D1. Proliferative index by Ki-67 stain is ~80%). Fluorescence in situ hybridization (FISH) analysis demonstrated rearrangements of BCL2/18q (80%) and MYC/8q (82%). The patient had a diagnosis of high-grade B-cell lymphoma with MYC and BCL-2 gene rearrangements. The second case is a 43-year-old woman with abdominal pain. She was diagnosed with acute cholecystitis and underwent cholecystectomy. Peritoneal fluid showed numerous neoplastic cells with irregular nuclei, prominent nucleoli, and cytoplasmic vacuoles. Flow cytometry demonstrated CD10-positive B-cells with lambda restriction. The cells were positive for CD10, CD20, BCL-2, BCL-6, and PAX5, and negative for CD34, cyclin D1, HHV-8, and TDT. EBER ISH was negative. The proliferation index by Ki67 stain was &gt;90%. FISH studies showed MYC, BCL2 and BCL6 gene arrangements. Cytogenetic analysis revealed a complex karyotype. A diagnosis of HGBL with triple-hit rearrangements was rendered. Despite receiving aggressive chemotherapy, the patient expired 8.5 months after the diagnosis.

Results (if a Case Study enter NA)
NA.

Conclusion
HGBL is an aggressive and often stage IV disease. It should be considered in the differential diagnoses of malignant effusions. Flow cytometry and FISH analysis of the body fluid specimen are essential to reach an accurate diagnosis in a timely manner.
Essential Thrombocythemia Associated with Plasma Cell Neoplasm
Authors: Eltahir M , Barbieri A , Rivero G 
Published: November 2022

Abstract

Introduction/Objective
The co-occurrence of essential thrombocythemia (ET) and multiple myeloma (MM), two distinct entities with distinct cellular origin, is rare, with a limited number of cases reported.

Methods/Case Report
We report a case of a 63-year-old male who initially presented with thrombocytosis, splenomegaly and elevated LDH. A bone marrow examination showed hypercellularity (90%) with increased abnormal megakaryocytes, as well as a JAK2 V617F mutation, with overall features consistent with involvement by a myeloproliferative neoplasm (MPN), consistent with ET (versus early primary myelofibrosis (PMF)). Additionally, 6% kappa-restricted plasma cells were identified, consistent with involvement by a monoclonal gammopathy of undetermined significance (MGUS). The patient was subsequently treated with hydroxyurea. Four years later, he presented with evidence of paraproteinemia. IgG kappa monoclonal paraprotein was elevated at 3.4 g/dL. A repeat bone marrow examination showed hyper-cellularity (60%), including clusters of abnormal megakaryocytes and mild to moderate reticulin fibrosis. The previously identified kappa-restricted plasma cell population increased to approximately 40% of the total cellularity. Cytogenetic analysis showed a normal male karyotype (46,XY[20]), and a prognostic myeloma-FISH panel including 13q-/-13, 1q32/1q21, p53/NF1, CCND1/IgH t(11;14), FGFR3/IgH t(4;14), IgH/MAF t(14;16) and IgH/MAFB t(14;20) was negative for all tested abnormalities. The overall features were again consistent with involvement by an MPN and progression of the previously identified MGUS.

Results (if a Case Study enter NA)
NA.

Conclusion
Only a few cases of concurrent ET and MM have been previously reported in the literature, with most of these cases having a temporal association with alkylating agent therapy. However, MM development has also been reported in a patient with non-cytotoxic treatment of ET. In contrast, our patient was diagnosed with ET associated with MGUS at the initial diagnosis. Notably, the co-existence of early PMF with MM appears to be relatively more established. A large study showed that previous PMF was strongly associated with MM development (OR 24.3; 95% CI:2.9-201.5).
Case Series: Large B-cell Lymphoma with IRF4 Rearrangement Involving the Lung and the Tonsil in Two Different Patients
Authors: Abdulrahim A 
Published: November 2022

Abstract

Introduction/Objective
Large B-cell lymphoma (LBCL) with IRF4 rearrangement is a new entity described in the 2017 WHO classification. It is characterized by a monotonous proliferation of medium to large sized cells with strong expression of IRF4/MUM1 and IRF4 rearrangement. It accounts for only 0.05% of all diffuse LBCLs and affects predominantly children and young adult with median age of 12 years. The reported sites of disease include lymph nodes of head and neck, Waldeyer ring and gastrointestinal tract. Here, we report two cases of Large B-cell Lymphoma with IRF4 rearrangement in two female patients, 73-year old and 71-year old respectively. The first patient presented with a lung nodule, while the second presented with right palatine tonsillitis. Hematoxylin and Eosin staining, immunohistochemistry and Fluorescence In-Situ Hybridization were used to characterize the lesions. H&E stained sections of the lung nodule biopsy from the first patient showed diffuse proliferation of medium- to large-sized cells (Figures A and B). Immunohistochemistry shows tumor cells are positive for CD20 (figure C), CD10, BCL6, and IRF4/MUM1(figure D). FISH study shows that tumor cells carry the IRF4 rearrangement but are negative for MYC, BCL2, and BCL6 translocations. To the best of our knowledge, this is the first case of LBCL with IRF4 rearrangement reported involving the lung in an older patient. H&E stained sections of the right palatine tonsil from the second patient shows effacement of the normal architecture which is replaced by sheets of large lymphoid cells. Immunohistochemistry shows tumor cells positive for CD20, CD10, BCL-6, BCL-2, MUM-1, C-MYC, and Ki-67 (80%). FISH study shows that tumor cells carry the IRF4 rearrangement which is consistent with a diagnosis of LBCL with IRF4 rearrangement.

Methods/Case Report
NA.

Results (if a Case Study enter NA)
NA.

Conclusion
NA.
Automated Blasts and Plasma Cells Counting on Manually Prepared Bone Marrow Aspirate Smears, Closest Deep Learning Pipeline for Generalizability
Authors: Elsafty A M , Ahmed Y M 
Published: November 2022

Abstract

Introduction/Objective
Medical errors, liability and reduced productivity in Hematopathology labs stem from fatigue, stress and shortage. 5-35% of complete blood count specimens (at least 3 billion tests worldwide annually) compared to 100% of bone marrow aspirates require visual examination using microscopes or digital scanners. The deep learning technologies and growing digital scanners market empower for developing work-fit automated visual examination to assist the Hematopathologist.

Methods/Case Report
2.6K+ manually smeared and stained bone marrow aspirate slides were scanned using integrated cameras on microscopes with electronic/mechanical control units. On top of the intracellular and background staining/dyeing variations, new scanning-related variations in exposure, resolution/focus and stitching were noted inter-patches and intra-patches. This led to consensus loss and "carry over" during cells identification. A convolutional autoencoder with skip connection for restoring/normalizing the stain and resolution was developed using data simulating the entire variations and artifacts. Generation of dimensions and ground truths was performed just by drawing on cells borders using pens, this enabled to localize and segment 5K+ cells per day with almost 100% accuracy. To help with labelling, intracellular colors split and quantification were applied. The processed 1.72+ million cells for 14 different WBCs classes/types were used to train a U-net architecture for segmentation, a transfer learning with pre-trained network EfficientNetB0 with ImageNet for classification and YOLO for detection. A previously developed pipeline for RBCs counting was used to select the proper areas for counting on smears. New diverse datasets were used for testing. Results were validated against confirmatory tests including immunohistochemistry and flow cytometry.

Results (if a Case Study enter NA)
The five-by-two cross validations revealed minor performance fluctuations (+/- 1.05%) with significant averages normalization SSIM, detection mAP, segmentation IoU, and classification F1-score exceeded 0.96, 0.97, 0.98 and 0.97, respectively. This beta prototype finds the proper areas for counting, calculates the M:E ratio, collects and counts the differential. Sufficient qualitative abnormalities assessment needs more work.

Conclusion
Without normalization and at least 100K cell images for each class, there is no generalizability.
A Case of Near Total Rh(D) Expression Loss in a Sickle Cell Patient Following Aplastic Anemia and Development of a Transient Auto-Anti-D
Authors: Sadough Shahmirzadi M , Lawicki S 
Published: November 2022

Abstract

Introduction/Objective
Red blood cell (RBC) antigens are inherited traits and, and their expression is consistent throughout the life of most individuals. Changes in RBC antigen expression rarely occur in association with hematologic malignancies. We report a case of Rh(D) antigen change in a patient with sickle cell disease (HbSS) after developing aplastic anemia (AA).

Methods/Case Report
Patient is a 21-year-old female with HbSS who developed progressively worsening anemia and severe thrombocytopenia over 1 month requiring frequent RBC and platelet transfusions. She was initially treated for idiopathic thrombocytopenic purpura with no significant improvement. Bone marrow examination demonstrated severe AA; additional viral, rheumatological, and hematologic studies were all non-informative. Subsequently, she was treated with corticosteroids, cyclosporin, anti-thymocyte immunoglobin and eltrombopag with minimal improvement. She has been non-compliant with maintenance cyclosporine and continued to be transfusion dependent in the past 2.5 years. She had always strongly typed 4+ Rh(D) positive at immediate spin (IS) before this event, but approximately about 1.5 years after the AA diagnosis her reactions became weaker (2-3+ at IS). She had a short-lived auto-anti-D identified on one occasion which has never been re-identified, but she has had very weak Rh(D) typings since this time with negative reactions at IS and only microscopically positive reactions at the anti-human globulin phase. The same pattern of reaction is present across a variety of commercial anti-D reagents. Molecular analysis reveals a normal RHD*01 hemi- or homozygous allele and no mutations known to cause a weak or partial Rh(D) type. No other antigen change is detected, and her extended phenotype matches a recently obtained genotype. She has been treated as both type O+ and O- in the last year by different facilities and transfused both types of RBCs without incident.

Results (if a Case Study enter NA)
NA.

Conclusion
We believe this is the first reported case of decreased Rh(D) expression following aplastic anemia and a transient auto-anti-D.
Effects of Storage Temperature and Duration on Flow Cytometry Analysis of Bone Marrow Aspirate Samples
Authors: Chen C , Cevallos S , Zhou Y 
Published: November 2022

Abstract

Introduction/Objective
Multiparameter flow cytometry immunophenotyping is a powerful and indispensable tool in the diagnosis of hematologic malignancies, but its accuracy and precision are vulnerable to pre-analytical interferences. Acid-citrate-dextrose (ACD) is one of three main anticoagulants used for marrow collection. It offers better preservation and is commonly used for apheresis stem cell collection. However, ACD may alter the pH of short-draw samples. It is unclear how prolonged storage in ACD affects the results of flow cytometry testing. In this pre-analytical study, we characterized the effects of temperature and storage duration in bone marrow aspirate collected in ACD.

Methods/Case Report
Five bone marrow specimens were collected in ACD and stored at 4°C or room temperature (RT), respectively. Aliquots of specimens were tested at 24, 48, and 72 hours. A flow cytometry panel including CD19, CD33, CD34, CD38, CD45, CD138, and 7-AAD was used to identify lymphocytes, monocytes, granulocytes, myeloid blasts, hematogones, and plasma cells. Absolute cell count, percentage of leukocytes, percentage of membrane- compromised cells (7-AAD bright), and mean-fluorescent-intensities were measured for each subset. The results of each storage condition were compared to the results on the day of sample collection.

Results (if a Case Study enter NA)
We found that granulocytes and plasma cells are most vulnerable to delayed testing, especially when stored at RT. Myeloid blasts, hematogones, monocytes, and lymphocytes were largely stable within 48 hours at 4°C or RT, but their relative representation in total leukocytes was affected by the diminished granulocytes. Variations in surface marker expression of CD45, CD38, and CD138 in the plasma cell subset were also noticed. This effect was less profound at 4°C than RT.

Conclusion
We conclude that delayed testing of specimens collected in ACD within 72 hours does not significantly impact the results of flow cytometry testing for the commonly studied subsets, and 4°C may be a more robust and preferable method for storage.
“You Get What You Need”: Early Results of BloodTrack System on Reducing Inefficiencies in Blood Product Allocation
Authors: Hung S , Woddor N , Husami S , Fernandez X 
Published: November 2022

Abstract

Introduction/Objective
The preparation of blood products allocated for massive transfusion protocol (MTP) activation and surgical cases consumes much time and energy of blood bank technologists. Often, these blood products are returned to the blood bank unused. Clinicians may activate the MTP preemptively to ensure the availability of blood products in case they are needed. Most blood products ordered for surgeries are placed on hold and not transfused. These returned units require additional inspections before they can be made available again. To reduce these inefficiencies, we implemented the BloodTrack [Haemonetics, Boston, MA] system, wherein blood products are stored in remote refrigerators and made available to clinicians near the patients’ bedside. Besides safeguarding units placed on hold for surgeries, we hypothesized that BloodTrack would provide greater visibility of available products in the emergency department (ED), thus reducing the rate of preemptive MTP activations.

Methods/Case Report
From March 2022 onwards, BloodTrack refrigerators with OnDemand software were active in the ED, operating room (OR), and two intensive care (ICU) units. We educated ED, OR, and ICU nurses on the appropriate use of BloodTrack. MTP activations were recorded from August 2021 to June 2022. Data on units allocated to OR patients by BloodTrack were collected from software-generated logs. Data on units allocated to OR patients by BloodTrack were collected from software-generated logs.

Results (if a Case Study enter NA)
Since its introduction in March, BloodTrack has dispensed 92 red blood cell units, which represents 12% of the total 766 units assigned. The remainder of 674 units were unused, and represent substantial savings in time otherwise spent re-instating them. Moreover, the rates of preemptive MTP activations trended downwards, from 28% (32/113) in the 7 months prior to implementation, to 13% (3/24) after implementation.

Conclusion
In conclusion, our implementation of BloodTrack has led to reduced MTP activations, improved efficiency of our blood product allocation process, and developed interdisciplinary care team integration.
Review of Effective Use of the Departmental Electronic Occurrence System for the Patient Care Related Safety Events. A CMC VAMC Experience
Authors: Patel S , Jhala D 
Published: November 2022

Abstract

Introduction/Objective
Electronic patient safety event reporting (e-reporting) is an effective mechanism to learn from errors and enhance patient safety. The Pathology and Laboratory Medicine (P&LMS) has established an electronic occurrence reporting (EOR) program to detect occurrences in patient results prior to the release of results, procedures for the documentation of corrected results that have been released, and any deviation to standard operating procedures. Patient safety and its monitoring is improved by using the EOR reporting system in which healthcare workers can document and learn from incidents, and thus prevent potential medical errors. The authors explore the use of the EOR in the calendar year 2021 and its effective use.

Methods/Case Report
The EOR is available to all the employees of P&LMS. This EOR allows the recording of all information pertinent to the occurrence (including pre-analytical, analytical, and post analytical issues) and a variety of issues (i.e., safety events, notifications, recalls, correction of released results, changed, results, or any unexpected standard operating procedure deviation). The section supervisor will initiate an investigation including root causes and outcomes of the occurrence and propose corrective action, recommendations, and follow-up in the Occurrence Detection Database. This is also reviewed at the quality management meeting and by the sectional pathologist director. For quality assurance review, the EOR’s usage for January 1st, 2021, to December 31st, 2021, was reviewed.

Results (if a Case Study enter NA)
The EOR successfully allowed the full documentation of all 870 reported occurrences during the study period in an organized fashion allowing appropriate tracking and investigation for quality improvement. All reported occurrences were handled appropriately with organized data to ensure quality assurance and improvement.

Conclusion
The EOR is a highly effective, successful program in terms of monitoring and assisting in ensuring timely action is taken in real time for patient care. Electronic reporting and documentation of incidents should be well supported by continuous staff training, help, and feedback. The ERO plays an important role in the maintenance of Veteran Affairs’ position as a High Reliability Organization (HRO).