Hematology Commons^™

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 …

Lymphoma Imaging In Hiv And Non-Hiv Patients: A Retrospective Cross-Sectional Study With Clinical And Pathological Correlation, Poonamjeet Loyal, Edward Chege, Jasmit Shah, Anne Mwirigi, Samuel Gitau

Imaging & Diagnostic Radiology, East Africa

Background: Patients with Human Immunodeficiency Virus (HIV)have an atypical imaging pattern of lymphoma. There is paucity of literature on differences in tumor volume or burden of disease amongst HIV positive patients compared with HIV negative patients and how this correlates with clinicopathological parameters of aggressiveness and prognosis.

Methods: This was a retrospective cross-sectional study of patients with non-Hodgkin lymphoma which were categorized into HIV positive and HIV negative. The tumor burden, disease sites, international prognostic score and Ki-67 index were recorded. Continuous variables were analyzed using the Kruskal Wallis test and categorical variables with Fisher’s Exact test.

Results …

Paroxysmal Cold Hemoglobinuria Associated With Monoclonal B-Cell Lymphocytosis In An Elderly Patient: A Report Of A Rare Case., Summiya Nasim Md, Matthew Bartock Do

PCI Publications and Projects

We report the case of an 86-year-old male with chronic anemia who presented with indirect hyperbilirubinemia, undetectable haptoglobin, and reticulocytosis. The peripheral smear showed no schistocytes. The cold-agglutinin titer was mildly positive, and the Donath-Landsteiner antibody test was positive. Bone marrow biopsy revealed a small CD5⁺ B-cell clone consistent with monoclonal B-cell lymphocytosis (MBL). The patient was diagnosed with paroxysmal cold hemoglobinuria (PCH) associated with MBL and treated with rituximab, achieving clinical stability without transfusion requirement. This case illustrates a rare coexistence of PCH and MBL in an elderly patient and emphasizes the importance of comprehensive hematologic evaluation for unexplained …

Upper Respiratory Tract Infection Leading To A New Diagnosis Of Sweet Syndrome And Monoclonal Gammopathy Of Unknown Significance, Nidhi Rawat, Meron Gebrehiwot, Jason Raw, Jeyaprakash Ramachandran

HCA Healthcare Journal of Medicine

Background

We present a rare case of Sweet syndrome with underlying monoclonal gammopathy of unknown significance (MGUS) which initially presented as upper respiratory tract infection.

Case Presentation

A 52-year-old woman presented with a complaint of sore throat for 6 days, productive cough and fever for 5 days, and red, pruritic, circular, tender rashes on face, arms and trunk for 2 days. There was a past history of similar self-limiting rashes presenting intermittently for 1.5 years. She also reported to be taking tablet ibuprofen, as required for the past 1-2 years, for cervical spondylosis. On integumentary examination, widespread, red, tender, annular …

Effect Of Valemetostat On The Pharmacokinetics Of Midazolam And Digoxin: A Phase 1 Drug-Drug Interaction Study In Patients With Non-Hodgkin Lymphoma, Masaya Tachibana, Steven Horwitz, Eric Jacobsen, Francine Foss, Pamela Allen, Pierluigi Porcu, Tatyana Feldman, Jia Ruan, Jonathan Brammer, Jie Wang, Shinichi Inaba, Yuka Iko, Keiko Nakajima, Yasuyuki Kakurai, Noriaki Kitami, Yang Chen, Yvonne Lau

Department of Medicine Faculty Papers

Valemetostat tosylate (valemetostat) is an oral, potent, dual inhibitor of enhancer of zeste homolog (EZH)2/1, under investigation in non-Hodgkin lymphomas (NHLs) and solid tumors. In vitro, it inhibits cytochrome P450 3A (CYP3A) and P-glycoprotein (P-gp) when combined with sensitive CYP3A or P-gp substrates. This drug–drug interaction (DDI) sub-study is part of the phase 1 trial of valemetostat monotherapy (DS3201-A-J101; NCT02732275), assessing the effect of valemetostat on the pharmacokinetics (PK) of sensitive CYP3A and P-gp substrates midazolam and digoxin, respectively, in patients with relapsed or refractory NHL, and its safety and efficacy. Patients received two simultaneous single doses of midazolam and …

Creation And Evaluation Of Standardized Electronic Health Record Templates For Transition Of Care In Sickle Cell Disease: A Pilot Study, Stephanie Howe Guarino, Mohan Madisetti, Payal C. Desai, Akshat Jain, Kenneth Rivlin, Jerome A. Osheroff, Robin Miller, Brandi Campanella, Sophie Lanzkron, Julie Kanter

Cardeza Foundation for Hematologic Research

Objective The objective of this pilot project was to create a standardized Transition Note in the electronic health record (EHR) to facilitate clinician communication during the transition process from pediatric to adult care for youth with sickle cell disease (SCD). Methods Using a modified Delphi process, consensus was reached among 78 member centers from the National Alliance of Sickle Cell Centers (NASCC) on the essential health data elements for a standardized EHR Transition Note. Templates were created in 2 different EHR systems and piloted at 2 SCD centers. Perceived clinician acceptability, appropriateness, and feasibility were assessed using the AIM-IAM-FAM measures …

Evaluating Anti-Sickling Therapies For Sickle Cell Disease: A Microfluidic Assay For Red Blood Cell-Mediated Microvascular Occlusion Under Hypoxia, Zoe Sekyonda, Yuxuan Du, Solomon Oshabaheebwa, Payam Fadaei, Yusang B. Ley, Calvin Abonga, Michael A. Suster, Pedram Mohseni, Umut A. Gurkan

Faculty Scholarship

Sickle cell disease (SCD) is characterized by the polymerization of hemoglobin S (HbS) upon deoxygenation, leading to the formation of sickled red blood cells (RBCs) with reduced deformability. Under hypoxic conditions, the impaired RBC behavior significantly contributes to vaso-occlusive events, hemolysis, and end-organ damage. Consequently, RBC deformability serves as a pivotal hemorheological biomarker for evaluating disease severity and therapeutic response. The OcclusionChip, a microfluidic assay, measures RBCs deformability through microcapillary occlusion. However, its current hypoxic assay relies on a complex nitrogen gas setup, rendering it bulky, expensive, and unsuitable for point-of-care diagnostic use. Here, we optimized a chemically induced hypoxia …

Growth Measurements In Ugandan Children With Sickle Cell Anaemia From A Hydroxyurea (Hydroxycarbamide) Treatment Trial Relative To Unaffected Sibling Controls, Dennis Kalibbala, Vincent Mboizi, Grace Nambatya, Susan Murungi, Joan Ashaba, Catherine Nabaggala, Lynnth Turyagyenda, Deogratias Munube, Phillip Kasirye, Robert Opoka

Paediatrics and Child Health, East Africa

No abstract provided.

Building Access To Care For Women And Girls+ With Bleeding Disorders: The Wgbd Clinic Of Excellence Model, Ming Y. Lim, Katherine C. Anguiano, Shannon L. Carpenter, Kerry B. Funkhouser

Manuscripts, Articles, Book Chapters and Other Papers

No abstract provided.

Artificial Intelligence In The Management Of Leukemia, Stephanie Koo, Austin P. Runde, Melvin Speisman

School of Medicine

BACKGROUND: Recently, given the demonstrated ability of AI to accurately characterize complex pathologies, AI has been proposed to be of use in the diagnosis, treatment, and monitoring of leukemias given their genetic complexity and subtype heterogeneity, array of treatments, and need for relapse detection. AI has several potential applications in the management of leukemia. First, it can be used to detect leukemia; using AI to detect nuances in lab values can ensure these deadly cancers are never missed. Second, AI can be used to risk-stratify patients and personalize treatments; leukemias are among the most genetically complex cancers with well-characterized risk …

Anemia Is Never The Diagnosis: Autoimmune Hemolytic Anemia As A Herald Of Late-Onset Systemic Lupus Erythematosus, Alhasan Asaad, Dixie Duran, Cristina Duran-Nesovic, Jose E. Campo Maldonado

Research Colloquium

Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder characterized by multisystem involvement and a highly variable clinical presentation. It predominantly affects women of childbearing age and arises from complex interactions between genetic, environmental, and hormonal factors, resulting in immune dysregulation and widespread inflammation. The disease can manifest in nearly any organ system, with common presentations including skin rashes, arthritis, renal dysfunction, and hematologic abnormalities. Despite advances in understanding its pathogenesis and management, SLE remains a diagnostic and therapeutic challenge due to its heterogeneous nature and potential for severe complications. This case report highlights a unique presentation of SLE, …

Platelet Transfusion Practices In The Icu: A Prospective Multicenter Cohort Study, Van Stefan, Raasveld Jorinde, Andrew Flint, Jimmy Schenk, Claudia Van Den Oord, Merijn Reuland, Sanne De Bruin, Jan Bakker, Maurizio Cecconi,, Marcus Lance

Anaesthesiology, East Africa

Objective: There is a lack of comprehensive international data regarding platelet transfusion practices in the ICU. This study aimed to evaluate the current occurrence rate of platelet transfusion in the ICU and provide an overview of platelet transfusion practices including indications for a platelet transfusion, thresholds, (non-)adherence and geo-economic region variations.

Design: International prospective cohort study.

Setting: Two hundred thirty-three centers in 30 countries worldwide.

Patients: All patients 18 years old and older, admitted to the ICU during a single study week, selected by each site from one of the 16 predefined weeks (March 2019 to …

Isatuximab Plus Bortezomib, Lenalidomide, And Dexamethasone For Transplant-Ineligible Newly Diagnosed Multiple Myeloma Patients: A Frailty Subgroup Analysis Of The Imroz Trial, Salomon Manier, Meletios-Athanasios Dimopoulos, Xavier P Leleu, Philippe Moreau, Michele Cavo, Hartmut Goldschmidt, Robert Z Orlowski, Muriel Tron, Christina Tekle, Marie-France Brégeault, Andrea T Shafer, Meral Beksac, Thierry Facon

Faculty, Staff and Student Publications

Patients with multiple myeloma (MM) meeting frailty criteria have worse outcomes than those identified as non-frail. Here, we present a post hoc subgroup analysis of IMROZ, a global, phase III, open-label study investigating isatuximab (Isa) with bortezomib, lenalidomide, and dexamethasone (VRd) followed by Isa-Rd (N=265) versus VRd followed by Rd (N=181) in newly diagnosed transplant-ineligible MM (Ti NDMM) patients using the simplified International Myeloma Working Group (sIMWG) frailty score. Although patients aged >80 years were excluded, there was no exclusion for patients meeting frailty criteria. All patients received standard VRd/Rd dosing; Isa-VRd patients received intravenous Isa (cycle 1, 10 mg/kg …

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 …

The Immunophenotypic And Genetic Characterization Of Pediatric T -L Ymphoblastic Leukemia With A Mature Immunophenotype, Mahsa Khanlari, Wei Wang, Parastou Tizro, Mohammad K Eldomery

Faculty, Staff and Student Publications

Not available.

Platelet Protease-Activated Receptor 4 Genotype And Response To Aspirin In Pregnancy, Rupsa C. Boelig, James V. Michael, Antonios Tawk, Tingting Zhan, Joanna S. Y. Chan, Walter K. Kraft, Steven E. Mckenzie

Department of Medicine Faculty Papers

The platelet protease-activated receptor 4 (PAR4) threonine 120 (Thr120) allele is an activating allele associated with reduced aspirin response in vitro. Aspirin is recommended in high-risk pregnancies to prevent preeclampsia and preterm birth. We evaluated the impact of PAR4 genotype on aspirin response in pregnancy, as measured by platelet function assay 100 (PFA-100) epinephrine closure time, and perinatal outcomes. We conducted a prospective cohort study of high-risk pregnant patients who took 81-mg aspirin daily. PFA-100 was assessed at baseline, 2 to 4 weeks after aspirin initiation (follow-up 1), and 28 to 32 weeks’ gestation (follow-up 2). Primary outcome was difference …