Hemic and Immune Systems Commons^™

Presentation, Diagnosis, And Acute Treatment Of Secondary Hemophagocytic Lymphohistiocytosis: A Case Report, Alexandra Bartholomew, Michael Connick, Catherine Loehr, Shane Sanne

School of Medicine Faculty Publications

Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare, immunologically driven disorder with a high mortality rate. It is typically diagnosed on the basis of clinical and laboratory criteria. We present the case of a 53-year-old woman with multiple previous hospitalizations for fever of unknown origin and fatigue. Her workup ultimately led to a diagnosis of HLH. We discuss the presentation, diagnostic criteria, and clinical treatment of secondary HLH to guide workup and management for future patients affected by this rare disease.

A Complex Case Of A Large Fibroid Uterus Compressing The Inferior Vena Cava Causing Recurrent Deep Vein Thrombosis And Pulmonary Embolisms, Jessica Gil, Jessica Klingensmith, Lauren Hendrix, Patrick J. Stocker, Olugbenga Oyesanmi

HCA Healthcare Journal of Medicine

Background

Uterine fibroids have been found to increase the risk for venous thromboembolism. There have been other case reports linking large uterine fibroids, pulmonary embolisms, and deep vein thrombosis (DVT) together due to compression of blood vessels and increased venous stasis. This case report will discuss a case of a large fibroid uterus compressing the inferior vena cava causing a patient with few risk factors to have recurrent DVTs and pulmonary embolisms.

Case Presentation

The patient was a 35-year-old woman who presented with repeated unprovoked thrombosis episodes. The patient had a full workup after the first episode of DVT and …

Imaging Hallmarks Of Idiopathic Intracranial Hypertension And Insights Into Pathogenesis, Albalooshy Basimah, Scott Faro, Hsiangkuo Yuan, Kiran Talekar, Prabath Mondel, Enchao Qiu, Joga Chaganti

Department of Radiology Faculty Papers

Idiopathic Intracranial hypertension (IIH), also referred to as pseudotumor cerebri, is a term used to describe increased intracranial pressure in the absence of a known identifiable secondary cause. Despite advancements of neuroimaging techniques, imaging of the pathological underpinnings in the diagnosis of IIH has been limited. Although the causation of IIH has been ascribed to increased Cerebrospinal Fluid production and disordered drainage through the dural sinuses, new evidence shows that the glymphatic system which is an alternate pathway of drainage is likely to play a pivotal role. In this review, we address the pathophysiological underpinnings in the causation of IIH …

Excess Fructose In High-Fructose Corn Syrup Reduces Cell Viability & Promotes Nitric Oxide Release In Raw 264.7 Macrophages, Renzo F. Galarza, Edward L. Villegas

Research and Creativity Symposium

High-fructose corn syrup (HFCS) is an artificial sweetener that contains slightly higher concentrations of fructose compared to standard sucrose. It has been linked to an increased risk of obesity, type 2 diabetes, cardiovascular disease, and fatty liver disease. While both HFCS and sucrose are widely used sweeteners, growing evidence suggests they may differ in their inflammatory potential. We sought to understand the effects of elevated fructose on RAW 264.7 macrophages. To investigate these effects, we treated the cells with either a sucrose solution or a
solution designed to mimic high-fructose corn syrup. Lipopolysaccharide (LPS) stimulation was used to model innate …

Igg4-Related Autoimmune Pancreatitis In Patients With Prior Occupational Exposure: A Case Report And Review Of Risks Concerning Blue-Collared Workers, Zachary A. Coren, Justin Le, Jason Tran, Nicholas Moscardelli, Krish Patel, Joshua Coren

Rowan-Virtua Research Day

Autoimmune pancreatitis (AIP), a manifestation of IgG4-related disease (IgG4-RD), is a rare, often misdiagnosed condition that can closely mimic pancreatic cancer, particularly in its clinical and radiographic presentation. We report the case of a 76-year-old retired plumber who presented with painless jaundice and was initially suspected to have pancreatic cancer based on imaging findings. Subsequent endoscopic evaluation, biopsy, and markedly elevated serum IgG4 levels led to a diagnosis of type 1 AIP. Treatment with corticosteroids resulted in rapid clinical improvement and reduction in IgG4 levels, confirming the diagnosis.

This case highlights the diagnostic challenges of AIP, especially among blue-collar workers …

Transcription Factor Runx1 Regulates Coagulation Factor Xiii-A (F13a1): Decreased Platelet-Megakaryocyte F13a1 Expression And Clot Contraction In Runx1 Haplodeficiency, Fabiola Del Carpio-Cano, Natthapol Songdej, Liying Guan, Guangfen Mao, Lawrence E. Goldfinger, Jeremy G.T. Wurtzel, Kiwon Lee, Michele P. Lambert, Mortimer Poncz, A. Koneti Rao

Cardeza Foundation for Hematologic Research

BACKGROUND: Germline RUNX1 haplodeficiency (RHD) is associated with thrombocy- topenia, platelet dysfunction, and predisposition to myeloid malignancies. Platelet expression profiling of an RHD patient showed decreased F13A1, encoding for the A subunit of factor (F)XIII, a transglutaminase that cross-links fibrin and induces clot stabilization. FXIII-A is synthesized by hematopoietic cells, megakaryocytes, and monocytes.

OBJECTIVES: o understand RUNX1 regulation of F13A1 expression in platelets/mega- karyocytes and the mechanisms and consequences of decreased F13A1 in RHD.

METHODS: We performed studies in platelets, human erythroleukemia (HEL) cells, and human CD34+ cell-derived megakaryocytes including on clot contraction in cells following small inhibitor RNA knockdown …

Neuroinflammatory Signaling And Immune Cell Infiltration Differ In Brains Of Rats Exposed To Space Radiation And Social Isolation, Austin M. Adkins, Zachary N. M. Luyo, Alea F. Boden, Riley S. Heerbrandt, Richard A. Britten, Laurie L. Wellman, Larry D. Sanford

Center for Integrative Neuroscience and Inflammatory Diseases (CINID) Faculty Publications

Astronauts on the proposed Mars missions will be exposed to extended periods of social isolation (SI) and space radiation (SR). SI and SR-induced immune dysregulation can result in persistent neuroinflammation and neuronal damage which could negatively impact an astronaut’s health and ability to maintain adequate levels of performance. The synergistic effects of combined SI and SR on immune system functionality and the brain remain unknown. Determining how single and combined inflight stressors modulate the immune system is crucial for fully understanding pathways impacting astronaut health and performance. We used ground-based analogs of SI and SR in rodent models to investigate …

Ancestry-Dependent Immunologic And Prognostic Effects Characterize The Prostate Cancer Urinary Proteome, Annie Ha, Jaron Arbet, Zhuyu Qiu, Amanda Khoo, Brian P. Main, Meinusha Govindarajan, Matthew Waas, Stanley K. Liu, O. John Semmes, Julius O. Nyalwidhe, Paul C. Boutros, Thomas Kislinger

Leroy T. Canoles Jr. Cancer Research Center Faculty Publications

Urine is an attractive biomarker analyte for non-invasive longitudinal monitoring of health and disease, particularly for diseases of the genitourinary tract, like prostate and bladder cancer. The composition of an individual's urine reflects both genetic and lifestyle characteristics that differ across geographies and populations, like diet, hydration and other socio-economic factors. While men of African ancestry have elevated prostate cancer risk, it is unclear to what extent this influences urinary biomarkers. We therefore quantified the urinary proteomes of 329 localized prostate cancer patients: 135 self-identifying as White and 194 self-identifying as Black. We identified 110 proteins that significantly differed between …

Large-Bore Mechanical Thrombectomy Versus Catheter-Directed Thrombolysis In The Management Of Intermediate-Risk Pulmonary Embolism: Primary Results Of The Peerless Randomized Controlled Trial, Wissam A. Jaber, Carin F. Gonsalves Md, Stefan Stortecky, Samuel Horr, Ripal T. Gandhi, Keith Pereira, Jay Giri, Sameer J. Khandhar, Khawaja Afzal Ammar, David M. Lasorda, Brian Stegman, Lucas Busch, David J. Dexter, Ezana M. Azene, Nikhil Daga, Fakhir Elmasri, Chandra R. Kunavarapu, Mark E. Rea, Joseph S. Rossi, Joseph Campbell, Jonathan Lindquist, Adam Raskin, Jason C. Smith, Thomas M. Tamlyn, Gabriel A. Hernandez, Parth Rali, Torrey R. Schmidt, Jeffrey T. Bruckel, Juan C. Camacho, Jun Li, Samy Selim, Catalin Toma, Sukhdeep Singh Basra, Brian A. Bergmark, Bhavraj Khalsa, David M. Zlotnick, Jordan Castle, David J. O'Connor, C. Michael Gibson, For The Peerless Committees And Investigators

Department Surgery Faculty Publications

BACKGROUND: There are a lack of randomized controlled trial data comparing outcomes of different catheter-based interventions for intermediate-risk pulmonary embolism.

METHODS: PEERLESS is a prospective, multicenter, randomized controlled trial that enrolled 550 patients with intermediate-risk pulmonary embolism with right ventricular dilatation and additional clinical risk factors randomized 1:1 to treatment with large-bore mechanical thrombectomy (LBMT) or catheter-directed thrombolysis (CDT). The primary end point was a hierarchal win ratio composite of the following (assessed at the sooner of hospital discharge or 7 days after the procedure): (1) all-cause mortality, (2) intracranial hemorrhage, (3) major bleeding, (4) clinical deterioration and/or escalation …

Anaplastic Large Cell Lymphoma In Children And Adolescents, Eric J. Lowe, Wilhelm Woessmann

Department of Pediatrics Faculty Publications

Anaplastic lymphoma kinase (ALK)-positive anaplastic large-cell lymphoma (ALCL) accounts for >95% of ALCL cases in children and adolescents. The first description of ALCL as a CD30-positive lymphoma in 1985 was followed by the detection of chromosomal translocations involving the ALK gene at chromosome 2p23. The pathogenesis of ALK-positive ALCL is based on signalling from the constitutive active ALK kinase. The clinical characteristics, therapy regimens and outcome data were reported in the 1990s and 2000s. Different chemotherapy regimens led to astonishingly similar long-term event-free survival rates of 70%, independent of the drugs, doses and duration of therapy. Additionally, patients with relapsed …

A Linear Presentation Of Cutaneous Rosai Dorfman Disease, Emily Sheetz, Ryan C. Saal, Molly K. Smith, Rachel Chikowski Byrd

Department of Medicine Faculty Publications

[Case description] A 53-year-old female with type II diabetes and hypertension presented to the dermatology clinic with a 3-month history of an asymptomatic linear eruption along the right knee that extended distally along the shin. Initial biopsy showed an atypical lymphohistiocytic infiltrate (Fig 1), which was found to be polyclonal. At her 3-month follow up visit, the eruption demonstrated significant clinical progression with numerous indurated papules and papulonodules, some of which were tender, along the right knee and pretibial surface (Fig 2). No improvement was noted with topical triamcinolone prescribed at her initial visit.

Sarcoidosis With Isolated Osseous Manifestations Mimicking Metastatic Malignancy, Ahmed Ismail, Ahmed Saleh, Yanis Boumber

Department of Medicine Faculty Publications

Sarcoidosis is an inflammatory disease of unknown etiology that commonly involves multiple organ systems, with the lungs and lymph nodes most frequently affected. Osseous involvement, although recognized, is considered uncommon and often overlooked due to its nonspecific clinical and radiographic features. Patients may be asymptomatic or present with pain, swelling, and functional limitations of the affected limb. Imaging can reveal findings such as bone lesions that mimic malignancy, including lytic or sclerotic changes. Diagnosis relies on histopathological confirmation of non-caseating granulomas. Treatment is directed at symptom relief and preservation of limb function. Here, we present a rare case of isolated …