Hematology Commons^™

Severity And Duration-Dependent Aortic Stiffening In A Rabbit Coarctation Model Identifies Constitutive Material Parameters As Promising Regional Biomarkers For Hypertension Progression, Arash Ghorbannia, Jamasp Azarnoosh, John F. Ladisa Jr.

VMASC Publications

Coarctation of the aorta (CoA) alters hemodynamics and drives regional remodeling, yet the influence of severity and duration on constitutive properties is undefined. We quantified mechanical responses from CoA to identify constitutive parameters capturing stiffening across clinical severities and durations. Mild (≤ 12 mmHg), intermediate (13–20 mmHg), and severe (> 20 mmHg) CoA was created in rabbits for short, long, and prolonged durations (~ 1, 3, or 22 weeks, respectively). Stress–stretch curves from proximal and distal regions were fit with multiple constitutive models (Linear Elastic, Neo-Hookean, Yeoh, Ogden, Mooney–Rivlin, Holzapfel). Model performance was evaluated by normalized RMSE and R², with …

Dupilumab In Chronic Rhinosinusitis Without Nasal Polyps: Randomized Phase 2 Trial (Orion), Stella E. Lee, Anju T. Peters, Philippe Gevaert, Joseph K. Han, Claus Bachert, Francisco Moreira Da Silva, Andrés Rosenblüt, Chih-Chi Hu, Jennifer Maloney, Paolo Caferra, Adrianna Michalak, Andrew P. Fontenot, Lacey B. Robinson, Neelam A. Phadke

Department of Otolaryngology (ENT) Faculty Publications

BACKGROUND: Chronic rhinosinusitis (CRS) is often driven by type 2 inflammation and is characterized by nasal obstruction/ discharge, facial pain/pressure, and/or reduced smell, either with or without nasal polyps (CRSwNP or CRSsNP).

OBJECTIVE: To test whether dupilumab improves radiographic features in CRSsNP. Methods: ORION (NCT04678856), a phase 2, randomized, multicenter, double blind, placebo-controlled study, assessed dupilumab efficacy and safety in adults with uncontrolled CRSsNP. Patients were randomized 1:1 to dupilumab or placebo for 24 to 52 weeks. Endpoints included changes from baseline at week 24 in Lund–Mackay computed tomography (LMK-CT) score (dupilumab only [primary] and vs placebo [secondary]), sinus Total …

Trends In Hypertension Prevalance And Control In The United States Over 25 Years, Omar Saleh, Neda Rehan, Saleh Mostafa, Rehan Qayyum

Department of Medicine Faculty Publications

Despite sustained public health efforts, hypertension remains highly prevalent in the United States, with persistent disparities across demographic and socioeconomic groups. We examined 25-year trends in hypertension prevalence, undiagnosed hypertension, and on-treatment uncontrolled hypertension in the United States using nationally representative National Health and Nutrition Examination Survey (NHANES) data from adults aged ≥18 years. Hypertension was defined as systolic BP ≥130 mmHg, diastolic BP ≥80 mmHg, or current antihypertensive use. Undiagnosed hypertension was defined as elevated BP without prior diagnosis or treatment, while on-treatment uncontrolled hypertension was defined as diagnosed hypertension with BP ≥130/80 mmHg despite treatment. Survey-weighted generalized linear …

Successful Use Of Chimeric Antigen Receptor T-Cell (Car-T) Therapy With Lisocabtagene Maraleucel In A Renal Transplant Recipient With Refractory/Relapsed Diffuse Large B-Cell Lymphoma (Dlbcl), Waqqas Tai, Fatima Chaudhry, Nesreen Shahrour, Jessica Thomas, Afoma Anyadibe, Anuoluwa Oyetoran, Swathi Gopishetty, Precious Idogun, Dilip Samarapungavan, Jassim H. Sarmad, Ishmael Jaiyesimi

Department of Medicine Faculty Publications

This case describes a 53-year-old male with end-stage renal disease who developed monomorphic post-transplant lymphoproliferative disorder (PTLD) in the form of diffuse large B-cell lymphoma (DLBCL) after kidney transplantation. Despite initial treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone (R-CHOP), the patient's disease progressed, and he was referred for chimeric antigen receptor (CAR) T-cell therapy with lisocabtagene maraleucel (liso-cel). Given his post-transplant status, his immunosuppressive agents (tacrolimus and mycophenolate) were held, and prednisone was tapered to 5 mg daily to maintain minimal baseline immunosuppression. After lymphodepleting chemotherapy and liso-cel infusion, the patient experienced no significant toxicities, including cytokine release syndrome (CRS) …

Selenium And Liver Steatosis And Fibrosis: Opposing Direct And Steatosis-Mediated Associations In A Large Cohort, Neda Rehan, Jubilee Benedict, Rehan Qayyum

Department of Medicine Faculty Publications

Background and Aims

Epidemiologic studies have reported conflicting associations between selenium exposure and liver disease. Whether selenium exerts opposing direct and steatosis-mediated effects on liver fibrosis is unknown. To evaluate the associations of blood selenium with elastography-defined hepatic steatosis and fibrosis and to determine whether hepatic steatosis mediates the relationship between selenium and fibrosis.

Methods

We conducted a cross-sectional analysis of adults aged >= 18 years in NHANES 2017-2023. Hepatic steatosis was assessed using the controlled attenuation parameter (CAP) and fibrosis using liver stiffness measurement (LSM). Blood selenium was categorized into quartiles. Survey-weighted multivariable linear regression and structural equation modeling …

Circulating Biomarker Results From A Phase 2 Study Of Seralutinib In Pulmonary Arterial Hypertension, Robin Osterhout, Athénaïs Boucly, Raymond L. Benza, Richard N. Channick, Kelly M. Chin, Robert P. Frantz, Anna R. Hemnes, Luke S. Howard, Vallerie V. Mclaughlin, Olivier Sitbon, Jean-Luc Vachiéry, Rotham T. Zamanian, Richard Aranda, Matt Cravets, Zhaoqing Ding, Thao Duong-Verlé, David Mattola, Robert F. Roscigno, Ravikumar Sitapara, Lawrence S. Zisman, Jean-Marie Bruey, Hossein-Ardeschir Ghofrani

Department of Medicine Faculty Publications

[Introduction] To the Editor: Pulmonary arterial hypertension (PAH) is a progressive disease characterized by obstructive pulmonary arterial remodeling (1). Seralutinib, an investigational inhaled tyrosine kinase inhibitor, potently and selectively targets kinases relevant to PAH pathobiology including platelet-derived growth factor receptors (PDGFR) α and β, colony stimulating factor 1 receptor (CSF1R), and mast/stem cell growth factor receptor kit (c-KIT) (2). In preclinical models, seralutinib improved cardiopulmonary hemodynamics, reversed pulmonary vascular pathology and decreased right ventricular hypertrophy (3). In TORREY, a phase 2, multicenter, double-blind, randomized, placebo-controlled trial in PAH, seralutinib significantly reduced pulmonary vascular resistance (PVR) after 24 weeks with good …

Cefazolin: A Rare Etiology Of Induced Coagulopathy, Ahmed Ismail, Anam Habib, Cayleigh Blumrick

Department of Medicine Faculty Publications

Cefazolin, a first-generation cephalosporin, is commonly used to treat methicillin-susceptible Staphylococcus aureus (MSSA) infections. Although generally well tolerated, coagulation abnormalities have rarely been reported during therapy. We describe the case of an 81-year-old man treated with prolonged cefazolin for MSSA endocarditis who developed severe coagulopathy characterized by marked elevation of both the international normalized ratio (INR) and activated partial thromboplastin time (aPTT). The abnormalities occurred after initiation of cefazolin and worsened despite cessation of other anticoagulants. Following discontinuation of cefazolin and administration of vitamin K, coagulation parameters rapidly improved and subsequently normalized. This case highlights a rare but clinically significant …

A Case Of Post-Transplantation Lymphoproliferative Disorder Following Kidney Transplantation, Punam Raval, John Odhiambo, Hanika Patel, Ahmed Sokwala

Internal Medicine, East Africa

This was a case of a 65-year-old gentleman known to have diabetes mellitus and hypertension since 2012 and post renal transplantation in 2016. He had also been treated for hepatitis C infection in the past. His regular medications included nebivolol 5 mg once a day, tacrolimus 4 mg twice a day, mycophenolate mofetil 500 mg twice a day, prednisolone 5 mg once a day, and insulin–novomix 14 units in the morning and 6 units at night. He presented to a tertiary teaching hospital in Kenya in October 2023 with abdominal pain, vomiting, and constipation on and off for 6 months, …

The Two-Step Allogeneic Stem Cell Transplant Is Associated With Excellent Engraftment, Improved Survival And Low Nonrelapse Mortality In Patients With Myelofibrosis, Xia Bi, Shaik Rashid, Joanne Filicko-O'Hara, Joseph Van Galen, William O'Hara, John L. Wagner, Dolores Grosso, Neal Flomenberg, Usama Gergis

Department of Medical Oncology Faculty Papers

Myelofibrosis is a chronic myeloproliferative disorder primarily affecting older adults, and allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative treatment option. However, HSCT for myelofibrosis presents significant challenges, including high nonrelapse mortality (NRM) and an increased risk of graft failure. In this 20-year retrospective study, we evaluated outcomes for 32 myelofibrosis patients who underwent HSCT at our institution, with particular focus on a two-step transplant approach designed to optimize T-cell dosing by separating the lymphoid and myeloid graft components, with cyclophosphamide administered after the lymphoid infusion and before infusion of CD34-selected stem cells. Eighteen patients underwent transplantation using …

Aggressive Natural Killer Cell Leukemia: A Rare And Rapidly Progressive Hematologic Malignancy—Case Report And Literature Review, Jennifer Priessnitz, Ali Hariri, Yuliya Levkiavska, Kyle E. Bonner, Stephan I. Fisher, Joshua M. Sill

Department of Medicine Faculty Publications

Aggressive natural killer cell leukemia (ANKL) is a rare, fulminant hematologic malignancy characterized by neoplastic proliferation of mature NK cells. It is frequently associated with Epstein-Barr virus (EBV) infection, although EBV-negative cases have also been reported. While typically observed in young to middle-aged adults of East Asian descent, increasing recognition has led to identification of ANKL across diverse age groups and ethnicities. The disease is defined by a rapid clinical course and poor prognosis, underscoring the importance of early diagnosis and effective treatment. We present a case of a 71-year-old Caucasian male who developed fever, altered mental status, hepatosplenomegaly, pancytopenia, …

Innovative Endovascular Stapling Technique Using The Aortoseal Device In Challenging Infrarenal Aortic Neck, Emidio Germano, Animesh Rathore, David J. Dexter

Department Surgery Faculty Publications

We report the use of the Aortoseal Endostapling system by Endoron in a 63-year-old man with infrarenal abdominal aortic aneurysm with high-risk neck features. Endovascular abdominal aneurysm repair was performed using a Medtronic Endurant IIs endograft with an adjunctive Aortoseal system as part of an international feasibility trial. The procedure was technically successful, with no endoleaks on completion angiography. Follow-up computed tomography angiography at 1 month confirmed a well-deployed AortoSeal system and complete aneurysm exclusion. This case highlights this innovative device and the technical considerations to enhance endograft proximal fixation with the potential to improve long-term outcomes in endovascular aneurysm …

Diagnosis Of Platelet Dysfunction In Children: Clinical Predictors And Test Methods, Bhavya S. Doshi, Eric N. Thompson, Walter Faig, Abigail Wax, Amrom E. Obstfeld, Michele P. Lambert

Student Papers, Posters & Projects

Evaluation for platelet function disorders (PFD) in children is complicated by their limited exposure to hemostatic challenges, large volumes needed for light transmission aggregometry (LTA) testing, and limited data on the performance characteristics of whole-blood methods such as whole-blood impedance lumiaggregometry (WBILA). The objective of this study was to determine the clinical variables associated with the diagnosis of a PFD. A single-center, retrospective, cohort study of children evaluated for PFD was conducted. Medical charts were abstracted for demographics, medications, testing indications, bleeding sites and severity, and laboratory results. Univariate odds ratios (OR) and multivariable modeling were conducted for association of …

The Breakdown Of Neurovascular Barriers: Molecular Mechanisms Of Tight Junction Dysfunction, Sowmya Shree Gopal, Mandeep Kaur, Sophie Lanzkron, Amit K. Srivastava

Department of Medicine Faculty Papers

The central nervous system (CNS) relies on tightly regulated barriers to maintain homeostasis and protect neural tissue from blood-borne toxins, pathogens, and inflammatory mediators. Tight junctions (TJs) are critical components of the blood-brain barrier (BBB) and blood-spinal cord barrier (BSCB), forming selective paracellular seals that regulate molecular trafficking. These structures comprise transmembrane proteins and cytoplasmic scaffolding proteins, which anchor TJs to the actin cytoskeleton. The spatial organization and function of TJs are dynamically regulated by calcium-dependent signaling, phosphorylation events, and G-protein-mediated pathways, which govern their assembly, disassembly, and response to physiological and pathological stimuli. The integrity of TJ complexes is …

G6pd Deficiency, Annette C. Osias Arredondo

Honors Capstones

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive enzyme disorder affecting over 400 million people worldwide.G6PD is essential for producing NADPH, which maintains reduced glutathione (GSH)—the RBC’s primary defense against oxidative damage. When G6PD activity is low, RBCs cannot neutralize oxidants, leading to hemoglobin oxidation, Heinz body formation, and subsequent bite cells and hemolysis. Symptoms range from asymptomatic to acute hemolytic crises, especially after exposure to oxidative triggers such as infections, certain drugs, or fava beans.

Orthogonal Comparison Of Nuclear And Mitochondrial Clonal Architectures In Hematologic Malignancies, Nehali Shah

Dissertations and Theses (Open Access)

Acute myeloid leukemia (AML) is a hematologic malignancy characterized by accumulation of mutations that disrupt hematopoietic differentiation and promote clonal expansion. Understanding how these mutations arise and evolve is essential for improving diagnosis, prognosis, and treatment stratification. Current methods are limited by either restricted genomic coverage (targeted panels) or low throughput and high cost (in single-cell whole genome sequencing, scWGS). An emerging alternative is the use of mitochondrial DNA (mtDNA) mutations as clonal markers.

This study aims to determine whether mitochondrial-derived clonal architectures correlate with nuclear-derived clonal architectures in AML, thereby evaluating mtDNA as a scalable orthogonal tool for lineage …

Ash Sickle Cell Disease Cpkd Guidelines Report, Robert I. Liem, Madeleine Verhovsek, Ted Wun, Sophie Lanzkron

Cardeza Foundation for Hematologic Research

No abstract provided.

Accidental Daily Dosing: Severe Methotrexate Toxicity In A Patient With Rheumatoid Arthritis, Sidra Memon, Kavitha Vemuri, Syed Uzair Mahmood, Darshit Patel

Advances in Clinical Medical Research and Healthcare Delivery

This case highlights a crucial complication of oral methotrexate therapy: toxicity due to dosing errors. A patient’s daily dosing regimen, instead of the intended weekly schedule, led to a significantly excessive cumulative dose of methotrexate over a short period, resulting in severe hematologic and mucosal toxicity. This underscores the importance of clear patient education and vigilant prescription practices to prevent such errors.

Plasmacytoma As The Initial Presentation Of Multiple Myeloma: A Case Report, Chantelle Martin, Sidra Memon, Austin Kim, Saleha Asghar

Advances in Clinical Medical Research and Healthcare Delivery

We report an unusual case of a woman who presented with nonspecific symptoms of unilateral facial mass and bleeding from her lower lip over several weeks. The mass was found to be relatively fixed and irregular with destruction of facial bone as evidenced by imaging. Initial investigations indicated the possibility of multiple myeloma, which was later confirmed with serum protein electrophoresis and bone marrow biopsy. Cervical lymph node biopsy confirmed presence of plasmacytoid cells positive for CD 20.

National Alliance Of Sickle Cell Centers Consensus Recommendations On Sickle Cell Disease Health Maintenance: A Consensus Statement, Seethal A. Jacob, Melissa Frei-Jones, Sana Saif-Ur-Rehman, Monica L. Hulbert, Andrew R. W. O'Brien, Crawford Strunk, Anthony Villella, Ravi Talati, Amma Owusu-Ansah, Francis Coyne, Patrick T. Mcgann, Parul Rai, Robin Miller, Angeli Rampersad, Vishwas Sakhalkar, Neha Bhasin, Deepa Manwani, Sophie Lanzkron, Julie Kanter

Cardeza Foundation for Hematologic Research

IMPORTANCE: The most common inherited blood disorder in the world, sickle cell disease (SCD) is a known global health concern. While survival of children into adulthood has improved significantly over the last few decades in high-resource countries, substantial gaps in care standards and limited practice harmonization persist.

OBJECTIVE: To provide up-to-date recommendations for preventive care and treatment of all people living with SCD and its related comorbidities.

EVIDENCE REVIEW: As part of the National Alliance of Sickle Cell Centers (NASCC), 41 pediatric and adult sickle cell disease experts developed consensus standards and recommendations based on available evidence and expert consensus …

Multinational Transfusion Practices And Outcomes In Haematology Patients Admitted To The Intensive Care Unit, Caroline Schaap, Laurens Oomen, Senta Raasveld, Jimmy Schenk, Sanne De Bruin, Merijn Reuland, Claudia Oord, Jan Bakker, Maurizio Cecconi, Marcus Lance

Anaesthesiology, East Africa

Background and Objectives The number of critically ill patients with haematological conditions is increasing, yet transfusion practices in this population remain poorly defined. This study aimed to compare transfusion strategies in critically ill patients with versus without haematological conditions.

Study Design and Methods This international, prospective observational substudy of the International Point Prevalence Study of Intensive Care Unit [ICU] Transfusion Practices (InPUT) evaluated transfusion use in ICU patients with and without haematological conditions, including benign or malignant diseases or a history of stem cell transplantation. Outcomes included use of red blood cells (RBCs), platelets, plasma, haemostatic interventions, transfusion indications and …