Hematology Commons^™

Successful Multimodal Interventional Management Of Extensive Portal Venous System Thrombosis In A Patient With A Hypercoagulable State, Dillon Woody, Jedediah Bondy, Donnette Dabydeen, Korinne Diss, Amanda Neider Neider

Advances in Clinical Medical Research and Healthcare Delivery

We present a case of extensive mesenteric and portal vein thrombosis in a 59-year-old male with a history of multiple thrombotic events and hypercoagulable conditions. The patient presented with abdominal pain, nausea, vomiting, and black stools, leading to the discovery of thrombosis in the portal vein, splenic vein, and superior mesenteric vein (SMV) with associated bowel ischemia. The goal of treatment was to prevent mesenteric ischemia, maintain portal vein patency, and preserve options for potential liver transplantation. The case was managed through a combination of ultrasound-guided paracentesis, portal and SMV venography, mechanical thrombectomy using the AngioJet device, and continuous thrombolytic …

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 …

Incidence Of Cytogenetic Abnormalities Detected By Fish Analysis In Multiple Myeloma Cases: A Seven-Year Study From A Cytogenetic Laboratory In King Chulalongkorn Memorial Hospital, Thailand (2018–2024), Montakarn Tansatit, Nutcharee Jongpornchai, Suwannee Songchart, Kittipornpan Krajokpap, Hudadini Da-Oh

Chulalongkorn Medical Journal

Background: Multiple myeloma (MM) is a genetically heterogeneous plasma cell malignancy with cytogenetic abnormalities influencing prognosis and treatment outcomes. Fluorescence in situ hybridization (FISH) is crucial for detecting clinically significant abnormalities, including IGH translocations and deletions (e.g., del(17p)), particularly in non-dividing plasma cells. However, cost and accessibility challenges limit comprehensive testing in resource-constrained settings like Thailand. Objectives: To investigate the incidence of cytogenetic abnormalities detected by FISH in MM cases over seven years in a Thai population, highlighting regional trends and barriers to comprehensive testing. Methods: A retrospective analysis was conducted on 360 bone marrow samples from MM patients between …

Outcomes And Microbiological Patterns Of Bacteremia In Chemotherapy-Related Febrile Neutropenia At A Tertiary Facility In Kenya, Abhijit Ghose, Jasmit Shah, Anne Mwirigi, Charles Makasa, Felix Riunga

Internal Medicine, East Africa

Introduction: Febrile neutropenia (FN) is a major cause of mortality and morbidity in patients with malignancy post-chmotherapy. Current guidelines for managing FN recommend starting empiric antibiotic therapy (EAT) promptly. The choice of antibiotic is based on the patient’s condition, local data on common microorganisms isolated, and their resistance patterns. Of note, there is a growing trend of isolating multi-drug–resistant gram-negative bacteria in these patients. In sub-Saharan Africa, there are insufficient data on the mortality rates of FN. There is also a lack of local epidemiologic data on the frequently isolated microorganisms and their antibiotic resistance patterns. This makes it challenging …

Antithrombotic Strategies And Outcomes In Neonates And Infants With Cardiac Shunts: A Systematic Review And Meta-Analysis., Amy L. Kiskaddon, Neil A. Goldenberg, Marisol Betensky, Joshua W. Branstetter, Dina Ashour, Pamela Williams, Arabela C. Stock, Michael Silvey, Therese M. Giglia, Nhue L. Do, Cardiac Disease Thrombosis And Hemostasis Working Party Of The Isth Ssc Subcommittee On Pediatric And Neonatal Thrombosis And Hemostasis

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Cardiac shunt thrombosis in neonates and infants remains a concern for shunt failure and mortality. The optimal strategy for thromboprophylaxis remains unknown.

OBJECTIVES: This systematic review aims to characterize antithrombotic strategies and outcomes in neonates and infants with a cardiac shunt.

METHODS: MEDLINE, Embase, and Cochrane CENTRAL were searched from inception through July 2024 for studies reporting shunt thrombosis prevalence among infants who received a cardiac shunt. We estimated the pooled prevalence of shunt thrombosis using random-effects meta-analysis. In the subgroup analysis, we evaluated the effects of shunt type and antithrombotic strategies on shunt thrombosis prevalence.

RESULTS: A total …

Mitophagy’S Impacts On Cancer And Neurodegenerative Diseases: Implications For Future Therapies, Jason Huang, Vincent Truong Pham, Shaozi Fu, Gang Huang, Ya-Guang Liu, Lei Zheng

Faculty, Staff and Student Publications

Substantial evidence supports an inverse relationship between cancer and neurodegenerative diseases (NDDs), but few studies investigate the biological mechanisms underlying this phenomenon. While previous explanations-such as inflammation, reactive oxygen species (ROS), genetic mutations, and cell death-remain significant, they ultimately converge on mitophagy. This review identifies mitophagy as a pivotal factor in the development of both cancer and NDDs, while also evaluating specific mechanisms and processes to clarify how mitophagy connects these opposing disease trajectories. By examining these factors, we aim to uncover the underlying mechanisms that explain the inverse relationship between cancer and NDDs, which will help develop therapeutic strategies …

Fractional Flow Reserve From Coronary Ct: Evidence, Applications, And Future Directions, Arta Kasaeian, Mohadese Ahmadzade, Taylor Hoffman, Mohammad Ghasemi-Rad, Anoop Padoor Ayyappan

Faculty, Staff and Students Publications

Coronary computed tomography angiography (CCTA) has emerged as the leading noninvasive imaging modality for the assessment of coronary artery disease (CAD), offering high-resolution visualization of the coronary anatomy and plaque characterization. The development of fractional flow reserve derived from CCTA (FFR-CT) has further transformed the diagnostic landscape by enabling the simultaneous evaluation of both anatomical stenosis and lesion-specific ischemia. FFR-CT has demonstrated diagnostic accuracy comparable to invasive FFR. The combined use of CCTA and FFR-CT is now pivotal in a broad range of clinical scenarios, including the evaluation of stable and acute chest pain, assessment of high-risk and complex plaque …

From Imaging To Computational Domains For Physics-Driven Molecular Biology Simulations: Hindered Diffusion In Platelet Masses, Catherine House, Ziyi Huang, Kaushik Shankar, Sandra Young, Meghan Roberts, Scott Diamond, Maurizio Tomaiuolo, Timothy Stalker, Lu Lu, Talid Sinno

Cardeza Foundation for Hematologic Research

When formed in vivo, murine hemostatic thrombi exhibit a heterogeneous architecture comprised of distinct regions of densely and sparsely packed platelets. In this study, we utilize high-resolution electron microscopy alongside machine learning and physics-based simulations to investigate how such clot microstructure impacts molecular diffusivity. We used Serial Block Face - Scanning Electron Microscopy (SBF-SEM) to image select volumes of hemostatic masses formed in a mouse jugular vein, producing high-resolution 2D images. Images were segmented using machine learning software (Cellpose), whose training was augmented by manually segmented images. The segmented images were then utilized as 2D computational domains for Lattice Kinetic …

Ptpn11 Mutations Define A Rare But Highly Adverse Subset Of Myelodysplastic Syndromes, Alexandre Bazinet, Alex Bataller, Guillermo Montalban-Bravo, Kelly Chien, Koji Sasaki, Wei Ying Jen, Mahesh Swaminathan, Tapan Kadia, Courtney Dinardo, Farhad Ravandi, Guillermo Garcia-Manero, Hagop Kantarjian

Faculty, Staff and Student Publications

No abstract provided.

Clinico-Epidemiological Characteristics Of Cerebral Venous Sinus Thrombosis In Kenya: A Retrospective Case Series, Taby Siika, Jaskirat Sokhi, Juzar Hooker, Sheila Waa, Anne Mwirigi, Jasmit Shah, Dilraj Sokhi

Internal Medicine, East Africa

Background: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke that is more common in young, especially female, adults and can be challenging to diagnose due to its frequently non-specific presentation and diverse risk factors. Most cases are idiopathic, and international guidelines do not recommend routine investigations for underlying thrombophilia. Timely diagnosis, with prompt neuroimaging and guideline-based treatment, leads to good outcomes. However, in the literature on CVST from sub-Saharan Africa, the gap is substantial, with the few cases described as being related to systemic and/or brain infections. We describe here the largest cohort of CVST from the …

Comparison Between Acute Leukemia Screening Tube And Lineage-Specific Panels For The Diagnosis Of Acute Leukemia In Kenya, Nancy Okinda, Geoffrey Omuse, Elizabeth Kagotho, Douglas Munga

Pathology, East Africa

Purpose: Acute leukemia is a group of hematologic malignancies categorized according to the immature cells that proliferate and replace the normal bone marrow. Flow cytometry has emerged as a cornerstone in the diagnosis of hematologic malignancies. Staged analysis with a screening tube containing specific lineage markers determines the need for subsequent testing if there is an abnormal population (blasts). The specific lineage panels to be analyzed are determined depending on the positive markers in the screening tube. This study aimed to determine the agreement of diagnosis using the acute leukemia screening tube (ALST) and the lineage-specific panel.

Methods: This was …

Risk Of Early Death After Acute Leukemia Diagnosis Among Adolescents And Young Adults, Amy M Berkman, Clark R Andersen, Vidya Puthenpura, Nicholas J Short, Kelly Merriman, Mahesh Swaminathan, Branko Cuglievan, David Mccall, Courtney Dinardo, Cesar Nunez, Nitin Jain, Tapan Kadia, Ghayas Issa, Amber Gibson, Miriam B Garcia, J Andrew Livingston, Susan Parsons, Michelle A T Hildebrandt, Michael E Roth

Faculty, Staff and Student Publications

Background: Advances in care have led to improvements in survival for adolescents and young adults (AYAs) diagnosed with cancer; however, the risk of early death remains high for certain cancers, particularly acute leukemias. Risk factors for early death in AYAs diagnosed with acute leukemia have not been well studied.

Methods: The Surveillance, Epidemiology, and End Results registry was used to assess risk of early death (within 2 months of diagnosis) in AYAs diagnosed with acute leukemia (n = 16 153). Early death proportion, by year, for AYAs diagnosed between 2006 and 2020 was described. Associations between incidence of early death …

Complications And Management Of Idiopathic Multicentric Castleman Disease, Kavanya Feustel, Jacob W. Keeling, Olivia Makos, Dmitriy Scherbak

HCA Healthcare Journal of Medicine

Background

The pathophysiology of idiopathic multicentric Castleman disease (iMCD) is poorly understood compared to the other subtypes of MCD, which has contributed to limited treatment options and poor prognosis for iMCD patients. The pathogenesis of iMCD is thought to be mediated in part by dysregulation of interleukin (IL)-6.

Case Presentation

We present a case report of a 39-year-old Caucasian man with siltuximab-refractory iMCD. He presented with severe lower extremity lymphedema and wounds. His disease progressed through standard-of-care siltuximab. Due to his severe disease-related morbidity, he contracted recurrent infections, often complicated by sepsis. Ultimately, he required a left lower extremity amputation. …

Immune Thrombocytopenic Purpura Association With Epstein-Barr Virus, Amer Aldamouk, Bachr Masri, Ali Khan, Pulkita Uppal

Graduate Medical Education Research Journal

Immune thrombocytopenia (ITP), also referred to as idiopathic thrombocytopenic purpura, is an acquired form of thrombocytopenia caused by antibodies directed against platelet glycoproteins, particularly anti-GpIIb/IIIa antibodies. Epstein-Barr virus (EBV), a herpes virus infecting a large majority of the population before adulthood, has been implicated in various immune-mediated disorders, including ITP. In this report, we present a case of a young adult female with a history of thrombocytopenia who was found to have severe thrombocytopenia attributed to chronic EBV infection. Despite initial treatment with corticosteroids and intravenous immunoglobulin (IVIG), the patient's condition necessitated second-line therapies, including rituximab and thrombopoietin receptor agonists. …

Cars And Trucks: Driving A Paradigm Shift In Hematologic Malignancies, Christine Charek, Tia Solh

Lynchburg Journal of Medical Science

Hematologic malignancies account for a significant number of annual cancer diagnoses and deaths across the globe. Historically, the prognosis for relapsed and/or refractory disease after standard therapies, such as chemotherapy and/or radiation, was poor. Chimeric antigen receptor (CAR) T-cell therapy offers an additional treatment option. While promising, this type of immunotherapy also comes with potentially severe side effects, toxicities, and limitations. A better understanding of the development, administration, and management of patients undergoing treatment can provide the general clinician with the knowledge to assist within a multidisciplinary team to ultimately improve patient outcomes.

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 Challenging Case Of Pyruvate Kinase Deficiency Complicated By Hemophagocytic Lymphohistiocytosis, Christopher Pope, Farah Ashraf, Jacqueline White, Akhilesh Sivakumar, Eric Behling, Tulin Budak-Alpdogan

Cooper Rowan Medical Journal

Pyruvate kinase deficiency (PKD) is an autosomal recessive disorder and the most common cause of chronic hemolytic anemia secondary to red blood cell (RBC) enzyme defects. Treatment is mainly supportive including splenectomy and transfusions. Mitapivat, an oral allosteric activator of defective pyruvate kinase, was approved by the FDA in February 2022 for treatment of adults with PKD. However, because Mitapivat undergoes liver metabolism, patients must have stable liver profiles prior to initiation of therapy. We present a case of PKD complicated by hemophagocytic lymphohistiocytosis (HLH). We initiated steroid therapy for his HLH with the intent to subsequently start Mitapivat. We …

Emerging Technologies Of Single-Cell Multi-Omics, Yi June Kim, Koichi Takahashi

Faculty, Staff and Student Publications

The heterogeneity of the hematopoietic system was largely veiled by traditional bulk sequencing methods, which measure the averaged signals from mixed cellular populations. In contrast, single-cell sequencing has enabled the direct measurement of individual signals from each cell, significantly enhancing our ability to unveil such heterogeneity. Building on these advances, numerous single-cell multi-omics techniques have been developed into high-throughput, routinely accessible platforms, delineating the precise relationships among different layers of the central dogma in molecular biology. These technologies have uncovered the intricate landscape of genetic clonality and transcriptional heterogeneity in both normal and malignant hematopoietic systems, highlighting their roles in …

Clinical Interrogation Of Tp53 Aberrations And Its Impact On Survival In Patients With Myeloid Neoplasms, Jayastu Senapati, Sanam Loghavi, Guillermo Garcia-Manero, Guillin Tang, Tapan Kadia, Nicholas J Short, Hussein A Abbas, Naszrin Arani, Courtney D Dinardo, Gautam Borthakur, Naveen Pemmaraju, Betul Oran, Elizabeth Shpall, Uday Popat, Richard Champlin, Sherry Pierce, Sankalp Arora, Ghayas Issa, Musa Yilmaz, Keyur Patel, Koichi Takahashi, Guillermo Montalban-Bravo, Danielle Hammond, Fadi G Haddad, Farhad Ravandi, Hagop M Kantarjian, Naval G Daver

Faculty, Staff and Student Publications

In myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML) with TP53 aberrations, dissecting the interaction amongst patient, disease and treatment factors are important for therapeutic decisions and prognostication. This retrospective analysis included patients with newly diagnosed MDS (>5% blasts) and AML with TP53 mutation(s) treated at MD Anderson Cancer Center. We factored patient age, TP53 aberration burden, therapy intensity and use of venetoclax in the AML subgroup, and allogeneic hematopoietic stem cell transplantation (HSCT) to interrogate outcomes. TP53 was annotated as high-risk (TP53HR) if >1 mutation, one mutation plus allelic deletion or a single mutation with variant allele frequency …

Increasing The Detection Of Pediatric Oncology Patients At Risk For Malnutrition Using A Specialized Screening Tool, Casey Treuting Md, Cori A. Morrison Md, Jourdan A. Mills Rd

Quality Improvement & Patient Safety Forum

No abstract provided.