Hematology Commons^™

Cns And Retinal Radiologic Findings Of A Young Patient With Heterozygous Prothrombin G20210a Gene Mutation, Justina Kasteri, Timothy Ehmann, Bryan Scott

Advances in Clinical Medical Research and Healthcare Delivery

Stroke is one of the leading causes of death and acquired long-term disability in the world.¹ In United States stroke is the 5^th leading cause of death with a mortality rate of 49.1 deaths per 100,000 people.² Strokes can be ischemic or hemorrhagic in origin, of which 85% are ischemic strokes. Approximately 10--15% of ischemic strokes occur in patients 18-50 years of age, and inherited thrombophilia may be a contributing factor through induction of a hypercoagulable state. Prothrombin G20210A mutation has an overall prevalence of approximately 2% of the general population, with an association between young patients …

Macular Thinning And Microvasculature Abnormalities In Children With Sickle Cell Disease: A Longitudinal Analysis, Sally S. Ong, Ann Nampomba, Sara Rahman, Loka Thangamathesvaran, Grace Reilly, Jianqiao Ma, Jay Vaidya, Sophie Lanzkron, Adrienne W. Scott

Cardeza Foundation for Hematologic Research

PURPOSE: To assess longitudinal changes in retinal thickness and vessel density (VD) in pediatric sickle cell disease (SCD).

DESIGN: A prospective cohort study.

PARTICIPANTS: Children (< 18 years old) with HbSS and HbS variant (HbSC and HbS thalassemia) genotypes were enrolled from a university-based retina subspecialty clinic from 2017 to 2019 and followed for ≥2 years.

METHODS: Participants received 3 × 3 and 6 × 6 mm OCT and OCT angiography scans at baseline and at each follow-up visit.

MAIN OUTCOME MEASURES: Retinal thickness, superficial capillary plexus (SCP), and deep capillary plexus (DCP) VD were compared over time.

RESULTS: Children with HbSS (n = 14) and HbS variant (n = 14) genotypes with ≥1 follow-up were included in the study (total 56 eyes). For HbSS, rates of retinal thinning per …

A Ticking Time Bomb: An Overview Of A Case Report Of Neutropenic Fever Secondary To Tick-Borne Illness, Yasemin Galiboglu, Danielle Thor, Joann Ha, Kristine Wong, Cindy Hou

Rowan-Virtua Research Day

The advent of immunomodulatory therapies and their ever-expanding number of treatment indications necessitates the understanding of their associated complications. Neutropenic fever serves as an example of these complications often encountered in clinical practice. Although neutropenic fever can result from virtually any pathogen, episodes of the syndrome secondary to tick-borne illness remain relatively undocumented in the scientific literature. In the case presented, a 77-year-old female with a pertinent past medical history of smoldering IgG multiple myeloma on active immunosuppressive therapy presented with a first-time episode of neutropenic fever likely secondary to tick-borne illness. Through this overview of a broader report, attention …

The Revised Cdc Chronic Pain Guidelines And Payer Opioid Analgesic Policies In Michigan, Victoria Tutag Lehr, Cynthia L. Arfken

Michigan Journal of Public Health

Abstract

The 2016 publication of A Guideline for Prescribing Opioids for Chronic Pain by the Centers for Disease Control and Prevention motivated commercial and Medicaid payers to restrict their opioid prescribing policies including the imposition of hard limits on opioid daily dosages and duration. One result of these policies was an increase in abrupt opioid discontinuation and rapid tapering events for patients receiving long-term opioid therapy. Responding to this misapplication of the 2016 guideline, the 2022 revised guidelines emphasized individualized opioid therapy and exclusion of certain chronic diseases. However, it is not known if the payers changed their policies to …

Nosebleed No More: Recognizing And Diagnosing Factor Viii Inhibitor, Salam Harb, Leidy Huie Martinez Md, Jose Alvarado Md, Ajit Koli Do, Hazem Nasef Ms4

HCA-NSU MD Research Day

Acquired hemophilia is a condition characterized by the unexpected onset of severe bleeding due to an issue with coagulation factors. Typically, this disorder results from the production of autoantibodies against the Factor VIII coagulation factor. Although acquired hemophilia is an extremely rare condition, with an annual incidence of just 1.5 cases per million, it is crucial to recognize and understand it. This is because diagnosing this disorder is intricate, and if left undiagnosed and untreated, it can lead to high levels of morbidity and mortality. This case stands out as the condition typically targets older adults, with around 80% of …

Brief Review: Regional Anesthesia For Vaso-Occlusive Pain Crises, Oluwatomi Alade

Rowan-Virtua Research Day

Vaso-occlusive pain crisis occurs with obstruction of blood vessels from sickled red blood cells. This results in ischemic injury causing in pain. Acute vasoocclusive pain crisis is one of the most common reasons for patients with sickle cell disease to present to the hospital for medical attention. Acute treatment involves IV opioid therapy, non-opioid therapy, and IV hydration. There is a known lack of trust between a patient in acute pain and a provider in the emergency department (ED) and hospital secondary to stereotypes regarding pain seeking behavior. Here we discuss a case of vasoocclusive pain crisis refractory to opioid …

Case Report: A Case Of Ttp In The Ed, Brian F. Lim, Andrew Caravello, James A. Espinosa, Alan Lucerna

Rowan-Virtua Research Day

We report a case of a 54-year-old female who presented with mild shortness of breath, lower chest discomfort, fatigue, and weakness ongoing for several days and was diagnosed with thrombotic thrombocytopenic purpura (TTP). TTP is characterized by microangiopathic hemolytic anemia and thrombocytopenia due to either an inherited or immune-mediated reduction in von Willebrand Factor (VWF) cleaving protease ADAMTS13.

Patients presenting with non-specific symptoms is becoming increasingly common and initial bias could be to attribute symptoms to viral syndrome or upper respiratory tract infection. However, the differential for non-specific complaints is extensive and thorough review of labs and re-evaluations of patients …

Chilling Complications: A Case Of Covid-Associated Cold Autoimmune Hemolytic Anemia (Aiha), Julian Coz, Kishan B. Patel

Rowan-Virtua Research Day

Cold Agglutinin disease (CAD) also known as Cold Autoimmune Hemolytic Anemia (AIHA) is a form of autoimmune hemolytic anemia wherein cold agglutinins (IgM autoantibodies against red blood cell (RBC) antigens) bind during cold temperatures causing clinical symptoms related to RBC agglutination resulting to hemolytic anemia. Clinicians should recognize that Cold Agglutinin disease can be secondary to an underlying pathology such as COVID-19. Here we describe an unusual case of Cold Agglutinin Autoimmune Hemolytic Anemia which was diagnosed in the Emergency Department with the presence of COVID-19 and with a hospital course complicated by acute deep vein thrombosis (DVT) and bilateral …

A Rare Presentation: Intracranial Hemorrhage As A Symptom Of Acute Leukemic Transformation In A 23-Year Old Male, Kelsey M. Murray, Kishan B. Patel

Rowan-Virtua Research Day

This case highlights the urgency of considering acute leukemic transformation in young patients presenting with neurological deficits, emphasizing the importance of prompt evaluation and management to optimize patient outcomes. The case depicted is a tragic complication of Chronic Myeloid Leukemia (CML) and its acute blast crisis. Remarkably, the patient exhibited none of the typical constitutional symptoms associated with CML.

Profiling The Activity Of The Para-Caspase Malt1 In B-Cell Acute Lymphoblastic Leukemia For Potential Targeted Therapeutic Application, Firas M Safa, Terri Rasmussen, Lorena Fontan, Min Xia, Ari Melnick, Adrian Wiestner, Patricia Lobelle-Rich, Jan A Burger, Yara Mouawad, Hana Safah, Erik K Flemington, Nakhle S Saba

Faculty, Staff and Student Publications

B-cell acute lymphoblastic leukemia (B-ALL) remains a hard-to-treat disease with a poor prognosis in adults. Mucosa-associated lymphoid tissue lymphoma translocation protein 1 (MALT1) is a para-caspase required for B-cell receptor (BCR)-mediated NF-κB activation. Inhibition of MALT1 in preclinical models has proven efficacious in many B-cell malignancies including chronic lymphocytic leukemia, mantle cell lymphoma and diffuse large B-cell lymphoma. We sought to examine the role of MALT1 in B-ALL and determine the biological consequences of its inhibition. Targeting MALT1 with both Z-VRPR-fmk and MI-2 efficiently kills B-ALL cells independent of the cell-of-origin (pro, pre, mature) or the presence of the Philadelphia …

Case Of Rosai Dorfman Disease In A Patient With Newly Diagnosed Hodgkin Lymphoma, Liza Salloum, Jennifer Rojas Huen, Ricardo Serna, Alisha Valdez

Research Symposium

Background: Rosai Dorfman Disease (RDD, also known as sinus histiocytosis with massive lymphadenopathy) is a rare non-Langerhans cell histiocytosis, classified into nodal (classic) and extranodal disease. Most patients present with non-tender cervical lymphadenopathy, although other sites including the inguinal and axillary regions have been reported. Extranodal disease (~43% of cases) can involve any organ system. RDD is more common in children, but cases have been reported in patients in their 70s. The prognosis of nodal RDD can correlate with the number of nodal groups involved. Many cases can be managed only with observation, although surgery, chemotherapy, corticosteroids, and immunomodulators have …

A True Bloody Emergency: An Unusual Case Of Thrombotic Thrombocytopenic Purpura, Joshua M. Ninan

Research Symposium

Background: Thrombotic thrombocytopenic purpura (TTP) is a primary thrombotic microangiopathy that is classically characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA). Although rare with an annual incidence of 3.7 cases per one million adults, it is considered a true hematological emergency due to its fatality rate of almost 100% if appropriate treatment is not initiated immediately. This makes it vitally important to identify and treat patients with TTP, a task that becomes unusually challenging in the absence of the disorder’s other characteristically diagnostic clinical features such as mucosal bleeding, fever, or presence of schistocytes.

Case Presentation: A 30-year-old gentleman with …

A Previously Healthy Infant With Lemierre Syndrome In The Emergency Department: Case Report, Adeola Adekunbi Kosoko, Omoyeni O Clement

Faculty, Staff and Student Publications

INTRODUCTION: Lemierre syndrome (LS) is a rare condition with a high mortality risk. It is well described in older children and young adults involving bacteremia, thrombophlebitis, and metastatic abscess commonly due to Fusobacterium infections. Young, pre-verbal children are also susceptible to LS; thus, careful attention must be given to their pattern of symptoms and history to identify this condition in the emergency department (ED).

CASE REPORT: A 12-month-old previously healthy boy with a recent diagnosis of acute otitis media and viral illness presented to the ED with a complaint of fever. Additional symptoms developed at the head and neck and …

Effect Of Thromboprophylaxis On Clinical Outcomes After Covid-19 Hospitalization, Tracy Y Wang, Abdus S Wahed, Alison Morris, Lisa Baumann Kreuziger, John G Quigley, Gervasio A Lamas, Alexandra J Weissman, Jose Lopez-Sendon, M Margaret Knudson, Deborah M Siegal, Raj S Kasthuri, Andrew J Alexander, Lana Wahid, Bassel Atassi, Peter J Miller, Janice W Lawson, Bela Patel, Jerry A Krishnan, Nancy L Shapiro, Deborah E Martin, Andrei L Kindzelski, Eric S Leifer, Jungnam Joo, Lingyun Lyu, Annie Pennella, Brendan M Everett, Mark W Geraci, Kevin J Anstrom, Thomas L Ortel, Activ-4c Study Group

Faculty, Staff and Student Publications

BACKGROUND: Patients hospitalized with COVID-19 have an increased incidence of thromboembolism. The role of extended thromboprophylaxis after hospital discharge is unclear.

OBJECTIVE: To determine whether anticoagulation is superior to placebo in reducing death and thromboembolic complications among patients discharged after COVID-19 hospitalization.

DESIGN: Prospective, randomized, double-blind, placebo-controlled clinical trial. (ClinicalTrials.gov: NCT04650087).

SETTING: Done during 2021 to 2022 among 127 U.S. hospitals.

PARTICIPANTS: Adults aged 18 years or older hospitalized with COVID-19 for 48 hours or more and ready for discharge, excluding those with a requirement for, or contraindication to, anticoagulation.

INTERVENTION: 2.5 mg of apixaban versus placebo twice daily for …

Not Just A Pain: A Medical Simulation Case About Biased Communication And Osteomyelitis In Pediatric Sickle Cell Anemia, Adeola A Kosoko, Yakira R Alford, Karl A Upplegger, Gowri S Stevens

Faculty, Staff and Student Publications

Introduction

Biases in communication can be harmful to patient perceptions of care and the medical team's decision-making. Optimal communication must be taught and practiced similarly to the optimal management of the complex medical conditions associated with sickle cell disease (SCD). This simulation is designed to teach about biases, optimizing communication to and about a patient with SCD, and appropriately diagnosing and managing pediatric osteomyelitis as a complication of SCD.

Methods

We designed and implemented a simulation case targeting emergency medicine residents and fellows to raise awareness about biases associated with SCD care and the complication of osteomyelitis in children with …

Primary Sjögren's Syndrome Presenting As Cerebral Venous Thrombosis: A Rare Case, Mohan Sonu Chandra, Monisha G A, Ravi Kiran M

Faculty, Staff and Student Publications

Sjogren's syndrome is a late-onset, slowly progressing autoimmune disease characterized by the destruction of the exocrine glands by lymphocytic infiltration, resulting in dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). Sjögren's syndrome may be associated with various autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. We report a case of a 34-year-old female who delivered a live baby 20 days ago. She presented in a postictal state after two episodes of tonic-clonic movements of limbs with altered sensorium with a history of headache for seven days. Further evaluation revealed that the subject had a history of multiple …

Complexities And Complications Of Extreme Obesity, Haval Ali, Udit Naik, Michelle Mcdonald, Mohammad Almosa, Karen Horn, Alexis Staines, Louis Maximilian Buja

Faculty, Staff and Student Publications

Obesity is a common chronic disorder and has detrimental long-term consequences if left untreated. Herein, we report a case of a young lady who suffered from morbid obesity and many of its consequences, and we present a literature review of these complications. While the cause of obesity is multifactorial, the genetic component is particularly important in the pathophysiology of marked obesity. Resistance to Leptin is considered one of the main causes of obesity. There is a unique relationship between polycystic ovary syndrome and obesity, as observed in our case. Obesity is associated with cardiovascular and lung diseases such as heart …

Practical Considerations Of Dissolved Oxygen Levels For Platelet Function Under Hypoxia, Branden Kusanto, Andrew Gordon, Leigh Naylor-Adamson, Lloyd Atkinson, Charlie Coupland, Zoe Booth, Yusra Ahmed, Isabel M Pires, Graeme J Stasiuk, Roger Sturmey, Simon D J Calaminus, Mònica Arman

Faculty, Staff and Student Publications

Investigating human platelet function in low-oxygen environments is important in multiple settings, including hypobaric hypoxia (e.g., high altitude), sea level hypoxia-related disease, and thrombus stability. These studies often involve drawing blood from which platelets are isolated and analysed at atmospheric conditions or re-exposed to low oxygen levels in hypoxia chambers before testing. However, it remains unknown how the in vitro handling of the samples itself changes their dissolved oxygen concentration, which might affect platelet function and experimental results. Here, we prepared healthy donor platelet-rich plasma and washed platelet (WP) suspensions and exposed them to 2% oxygen. We found that the …

Thrombosis With Thrombocytopenia Syndrome After Administration Of Azd1222 Or Ad26.Cov2.S Vaccine For Covid-19: A Systematic Review, Usama Waqar, Shaheer Ahmed, Syed M H Ali Gardezi, Muhammad Sarmad Tahir, Zain Ul Abidin, Ali Hussain, Natasha Bahadur Ali, Syed Faisal Mahmood

Medical College Documents

Background: Cases of thrombosis with thrombocytopenia syndrome (TTS) have been reported following vaccination with AZD1222 or Ad26.COV2.S. This review aimed to explore the pathophysiology, epidemiology, diagnosis, management, and prognosis of TTS.
Methods: A systematic review was conducted to identify evidence on TTS till 4th September 2021. Case reports and series reporting patient-level data were included. Descriptive statistics were reported and compared across patients with different sexes, age groups, vaccines, types of thrombosis, and outcomes.
Findings: Sixty-two studies reporting 160 cases were included from 16 countries. Patients were predominantly females with a median age of 42.50 (22) years. AZD1222 was administered …

Strategic Priorities For Hematopoietic Stem Cell Transplantation In The Emro Region, Syed Osman Ahmed, Riad El Fakih, Alaa Elhaddad, Amir Ali Hamidieh, Abdulghani Altbakhi, Qamar-Un-Nisa Chaudhry, Ali Bazarbachi, Salman Adil, Murtadha Al-Khabori, Tarek Ben Othman

Section of Haematology/Oncology

The World Health Organization-designated Eastern Mediterranean region (EMRO) consists of 22 countries in North Africa and Western Asia with a collective population of over 679 million. The area comprises some of the wealthiest countries per capita income and some of the poorest. The population structure is also unique and contrasts with western countries, with a much younger population. The region sits in the heart of the thalassemia belt. Many countries have a significant prevalence of sickle cell disease, and cancer is on the rise in the region. Therefore, the strategic priorities for the growth and development of hematopoietic stem cell …

Occlusive Pulmonary Artery Thrombosis In A Healthy Neonate With No Identifiable Risk Factors, Cynthia Laviolette, Jason Turner, Latasha Lewis, Song-Gui Yang, Tim Pettitt, Kurt D Piggott

Faculty, Staff and Student Publications

Pulmonary artery thrombosis is reported in neonates with risk factors for hypercoagulability. No consensus exists regarding standard therapy for this condition. We present a neonate, with no risk factors for thrombosis, who was admitted after birth to the Pediatric Cardiac Intensive Care Unit with an occlusive left pulmonary artery thrombus.

The Effect Of Carfilzomib And Bortezomib Based Regimes On Cardiotoxicity In Multiple Myeloma Patients At Cooper University Hospital, Ami Patel, Tulin Budak-Alpdogan, Stalam Tapati

Rowan-Virtua Research Day

Introduction

• Multiple myeloma (MM) is a cancer of plasma cells, which is a white blood cell that normally produces antibodies
• Treatment in patients younger than 65 years old is typically high dose chemotherapy, usually with bortezomib based regimens or lenalidomide dexamethasone, followed by a stem cell transplant
• For patients with relapsed myeloma, carfilzomib is usually the treatment of choice
• Carfilzomib is a highly selective, irreversible proteasome inhibitor that binds to the 20 S proteasome. Several studies have illustrated that carfilzomib has been associated with cardiovascular adverse events (CVAE).
• Current literature on the role and effect of bortezomib on cardiotoxicity is …

Multiple Myeloma With Dual Expression Of Kappa And Lambda Light Chains, Monica Patel, Akash Patel, Yvette Wang

Rowan-Virtua Research Day

Multiple myeloma (MM) is a malignancy of plasma cells that accounts for approximately 1 to 2 percent of all cancers and about 17% of all hematologic malignancies.

Plasma cells normally produce antibodies and provide a defense mechanism for the body to fight infections.

Antibodies typically consist of two heavy chains (IgG, IgA, IgM, IgD and IgE) and two light chains (kappa and lambda).

Most cases of MM have malignant plasma cells producing monoclonal (M) proteins, most common being IgG about 52% of the time (1).

Only about 2% of these myeloma cases were also found to secrete more than one …

Metastatic Breast Cancer Presenting As Painless Jaundice, Daniel S. Wilen, Donald J. Mcmahon, Brian J. Blair, Joanne Kaiser-Smith, Darshan B. Roy

Rowan-Virtua Research Day

Breast cancer is the most commonly diagnosed cancer in women in the United States. Treatment options are wide-­‐ ranging based on a multitude of factors, including extent of disease and receptor positivity. Following treatment, a relapse-­‐free interval ≥ 2 years carries a more favorable prognosis.

We present a case involving a female with history of breast cancer and relapse-­free interval of 15 years who unfortunately presented with metastatic disease involving the liver, bone, and lymph nodes.

Classification And Immunophenotyping Of Acute Leukemias: A Prospective Study., Shahid Pervez, M. Khurshid

Department of Pathology and Laboratory Medicine

Over a period of 3-1/2 years, 86 cases of acute leukemia were analyzed by immunohistochemical (IHC) means on ficoll separated cytospin preparations of peripheral blood and/or bone marrow samples. Antibodies included in the panel were specific against Tdt, HLA-DR, CD19/CD2O/CD22, CALLA (CD1O), CD2, CD11C as well as against Ig heavy chains. Of 86 cases analyzed, 48 cases were of ALL, (25 of common pre-B ALL, 15 of pre-B/NULL and 8 of T ALL phenotype), twenty-four (24) out of 86 cases were of non­lymphoblastic (AML/AMML) type. In six cases, there was suggestion of a mixed lineage, while in 8 cases there …