Hematology Commons^™

A New Paradigm: Ecmo Therapy In Pediatric Hematology/Oncology Patients, Igor Areinamo

Research Days

Background: The use of extracorporeal membrane oxygenation (ECMO) for support of pediatric patients has significantly increased in the past years. Patients with known oncologic diseases, immunodeficiencies, or bone marrow transplants (BMT) have traditionally not been considered candidates for Extracorporeal membrane oxygenation (ECMO) due to perceived high risk for mortality. Ongoing advances have continued to push the boundaries of ECMO use and we report our recent experience with this patient population

Objectives/Goal: To evaluate survival to discharge of pediatric patients who require ECMO support and who also have an underlying hematologic or oncologic disease process. To determine specific patient characteristics that …

Bleeding Disorder Referrals To Hematology Clinic: A Single Institution Experience, Zuri Hudson

Research Days

Background: Our center receives hundreds of referrals yearly for bleeding disorder evaluation both due to bleeding symptoms and secondary to routine preoperative laboratory testing. The evaluation for a bleeding disorder can be challenging due to the wide variability of symptoms as well as the need for accurately interpreting lab results. Bhasin et al showed that 4% of patients referred to hematology based on a preoperative coagulation evaluation had a clinically relevant bleeding disorder. Currently there is little published about the referral patterns to pediatric hematology and the outcomes of these referrals.

Objectives/Goal: To characterize our hematology referrals for bleeding disorder …

Myocarditis Concurrent With Sweet Syndrome: A Presentation Of Acute Myeloid Leukemia, Christopher G. Burkeen, David Pottinger, Chaitanya Iragavarapu, Reshma Ramlal, Gerhard C. Hildebrandt

Markey Cancer Center Faculty Publications

Acute myeloid leukemia (AML) is the most common acute leukemia in American adults and portends a poor prognosis if untreated. Commonly, AML presents with symptoms related to concurrent leukopenia, anemia, or thrombocytopenia; however, due to its ability to affect many organ systems in the body, AML can have a highly varied clinical presentation. One such presentation is myocarditis, which is a rarely reported manifestation of AML. Myocarditis can have a varied clinical picture and often requires exclusion of other causes of cardiac dysfunction. Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is another presentation of AML; however, it is …

Type 1 Cryoglobulinemia Secondary To Primary Plasma Cell Leukemia, Antonio Faieta, Khatoon Tawhida

Conference Presentation Abstracts

Primary plasma cell leukemia (pPCL) is a rare and aggressive form of plasma cell dyscrasia that comprises 1% of the plasma cell dyscrasias. Even more rare is its association with type 1 cryoglobulinemia which management can be challenging. A 63-year-old African American male with no past medical history presented with severe burning pain in his legs, necrotizing rash, constitutional symptoms and severe acute kidney injury (AKI). HIV, hepatitis B and C were negative. Serum electrophoresis revealed an M-spike of 5.67, IgG of 7000, free lambda of 152.10 with a K/L of 0 and cryoglobulin of 81%. CT revealed extensive lytic …

Rare Case Of Recurrent Hyperhemolysis Syndrome (Hhs) And Methemoglobinemia In A Patient With Sickle Cell Anemia, Ashbina Pokharel, Pradeep Khanal, Dilip Khanal, Samikshya Pandey, Bipin Ghimire

Conference Presentation Abstracts

Hyperhemolysis syndrome (HHS) is a rare but serious and potentially life-threatening complication of red blood cell (RBC) transfusion and has been described in both sickle cell disease and non-sickle cell disease patients. It is characterized by destruction of both donor and host RBC. Methemoglobinemia is another rare condition leading to cyanosis, tissue hypoxia and potentially death. We present a middle-aged female with sickle cell anemia who developed methemoglobinemia and hyperhemolysis after a surgical procedure. 41 yo female with sickle cell anemia (SC type) was hospitalized for elective right total hip resection arthroplasty. Patient received 3 units blood transfusion during the …

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 …

Survival Benefit For Individuals With Constitutional Mismatch Repair Deficiency Undergoing Surveillance, Carol Durno, Ayse Bahar Ercan, Vanessa Bianchi, Melissa Edwards, Melyssa Aronson, Melissa Galati, Eshetu G. Atenafu, Gadi Abebe-Campino, Abeer Al-Battashi, Naureen Mushtaq

Section of Haematology/Oncology

Purpose: Constitutional mismatch repair deficiency syndrome (CMMRD) is a lethal cancer predisposition syndrome characterized by early-onset synchronous and metachronous multiorgan tumors. We designed a surveillance protocol for early tumor detection in these individuals.
Patients and methods: Data were collected from patients with confirmed CMMRD who were registered in the International Replication Repair Deficiency Consortium. Tumor spectrum, efficacy of the surveillance protocol, and malignant transformation of low-grade lesions were examined for the entire cohort. Survival outcomes were analyzed for patients followed prospectively from the time of surveillance implementation.
Results: A total of 193 malignant tumors in 110 patients were identified. Median …

Prospective Patient-Reported Symptom Profiles Associated With Pediatric Acute Lymphoblastic Leukemia Relapse, Austin L Brown, Kimberly P Raghubar, Olga A Taylor, Melanie Brooke Bernhardt, Lisa S Kahalley, Wei Pan, Philip J Lupo, Marilyn J Hockenberry, Michael E Scheurer

Faculty, Staff and Students Publications

PURPOSE: Despite improvements in frontline pediatric acute lymphoblastic leukemia (ALL) treatment, relapse remains a concern. Research in adult cancer patients suggests that patient-reported symptoms may predict survival, but the relationship between symptoms and relapse for pediatric ALL has received little attention.

METHODS: Pediatric patients with ALL (age 2-18 years) and/or their primary caregivers completed symptom surveys at the end of induction, start of delayed intensification (DI), start of maintenance cycle 1 (MC1), and start of maintenance cycle 2 (MC2). Symptom clusters for co-occurring fatigue, pain, sleep disruptions, and nausea were defined using latent profile analysis. Hazard ratios (HR) and 95% …

18f-Fluorothymidine Pet Is An Early And Superior Predictor Of Progression-Free Survival Following Chemoimmunotherapy Of Diffuse Large B Cell Lymphoma: A Multicenter Study, Ryogo Minamimoto, Luis Fayad, Julie M. Vose, Jane L. Meza, Ranjana Advani, Jordan Hankins, Felix Mottaghy, Homer Macapinlac, Alexander Heinzel, Malik E. Juweid, Andrew Quon

Journal Articles: Oncology and Hematology

PURPOSE: To determine whether interim 3'-deoxy-3'-[

METHODS: Ninety-two prospectively enrolled patients with DLBCL underwent both FLT-PET/CT and FDG-PET/CT 18-24 days after two cycles of R-CHOP/R-EPOCH. Deauville-criteria, PERCIST1.0, standardized uptake value (SUV), total lesion glycolysis (TLG), and metabolic tumor volume were used to interpret iFDG-PET/CT while dichotomous visual interpretation was used to interpret iFLT-PET/CT and the results were compared with the 3- and 5-year PFS.

RESULTS: iFLT-PET/CT was negative in 67 (73%) and positive in 25 (27%) patients. iFDG-PET/CT by Deauville criteria was negative (Deauville scores [DS] of 1-3) in 53 (58%) and positive (DS = 4-5) in 39 (42%) patients. …

Fall Prevention Using A Standardized K-Card Process, Falguni Shah Msn, Rn, Cms-Rn, Cnl, Anna Varghese Dnp, Msn, Rn, Ocn

Education Week Poster Showcase 2021

People's Choice winner in the 2021 Education Week Poster Competition

Department of Nursing

Department of Nursing Education

Genetic Correlates In Patients With Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia Treated With Hyper-Cvad/Hyper-Cmad Plus Dasatinib Or Hyper-Cvad Plus Ponatinib, Yuya Sasaki Md, Phd, Hagop Kantarjian Md, Nicholas J. Short Md, Farhad Ravandi Md, Marina Konopleva Md, Phd, Guillermo Garcia-Manero Md, Andrew Futreal, Feng Wang, Koichi Takahashi Md, Phd, Elias Jabbour Md

Education Week Poster Showcase 2021

Department of Leukemia Research

Department of Leukemia

Department of Genomic Medicine

Supply, Demand, And Quality: A Three-Pronged Approach To Blood Product Management In Developing Countries, Kyle L. Gress, Karina Charipova, Ivan Urits, Omar Viswanath, Alan D. Kaye

Journal of Patient-Centered Research and Reviews

While transfusion of blood and blood products is instinctively linked to the provision of emergent care, blood and blood products are also routinely used for the treatment of subacute and chronic conditions. Despite the efforts of the World Health Organization and others, developing countries are faced with a three-part problem when it comes to access to and delivery of transfusions: insufficient supply, excessive demand, and inadequate quality of available supply. Developing countries rely heavily on replacement and remunerated donors rather than voluntary nonremunerated donors due to concerns regarding donation- and transfusion-transmitted infection as well as local and cultural beliefs. While …

D-Dimer Levels And Acute Pulmonary Embolism Development In Covid-19 Patients, Vural Ahmet, Kahraman Ahmet Nedim

Journal of Mind and Medical Sciences

Objective. To identify those who develop pulmonary embolism with Ddimer levels by evaluating pulmonary CT angiographies of patients who are followed up with suspicion of coronavirus disease 2019 (COVID-19). Methods. Patients who were followed up in a community hospital with suspicion of COVID-19 and underwent Pulmonary CT angiography examination were evaluated. Clinical and demographic parameters and DDimer values for patients with and without pulmonary embolism were evaluated in the pulmonary CT angiogram. Results. During the COVID-19 pandemic, Thorax CT examination was performed in our center for suspicion or follow-up of COVID-19 infection in 3396 patients. Pulmonary CT angiography was applied …

Structure, Function And Inhibition Of Critical Protein-Protein Interactions Involving Mixed Lineage Leukemia 1 And Its Fusion Oncoproteins, Xin Li, Yongcheng Song

Faculty, Staff and Students Publications

Mixed lineage leukemia 1 (MLL1, also known as MLL or KMT2A) is an important transcription factor and histone-H3 lysine-4 (H3K4) methyltransferase. It is a master regulator for transcription of important genes (e.g., Hox genes) for embryonic development and hematopoiesis. However, it is largely dispensable in matured cells. Dysregulation of MLL1 leads to overexpression of certain Hox genes and eventually leukemia initiation. Chromosome translocations involving MLL1 cause ~ 75% of acute leukemia in infants and 5-10% in children and adults with a poor prognosis. Targeted therapeutics against oncogenic fusion MLL1 (onco-MLL1) are therefore needed. Onco-MLL1 consists of the N-terminal DNA-interacting domains …

Enhancing Medication Adherence Behaviors Among Ambulatory Pediatric Hematology & Oncology Patients: A Quality Improvement Project, Desiree Palmer

Doctoral Projects

Introduction: Researchers have found that pediatric patients with acute lymphoblastic leukemia and adolescent patients with sickle cell disease struggle to adhere to medication treatment requirements. Because of this trend, there is a clear need to address medication adherence. The purpose of this quality improvement project was to enhance medication adherence behaviors among pediatric hematology and oncology patients and their families within an ambulatory hematology and oncology clinic.

Methods: The project included a systematic literature review to identify evidence-based interventions, an organizational assessment, and a three-phase multifaceted quality improvement process. To increase patients bringing medications to appointments, 32 medication transportation bags …

Goals-Of-Care & End-Of-Life Quality In Relapsed High-Risk Leukemia: Silent Conversations, Lacy Jo Graham

Doctor of Nursing Practice Scholarly Project

BACKGROUND: The distorted association of “end of life (EOL)” with “goals of care (GOC)” has “silenced” crucial goals discussions in patients with relapsed high-risk leukemia, which raises concerns for the provision of care that is inconsistent with patient’s values and preferences.

AIM: The two main goals of this study were to quantify hematologists rate of participation in a GOC pathway initiative during two separate months, then explore their definition and barriers to having/documenting GOC discussions.

DESIGN: Mixed-methods, explanatory sequential design (follow-up explanations variant).

SAMPLE: Quan: Hematology inpatient admissions during the months of October 2020 and January …

Comorbidities And Complications In Adults With Pyruvate Kinase Deficiency., Audra N. Boscoe, Yan Yan, Elizabeth Hedgeman, Eduard J. Van Beers, Hanny Al-Samkari, Wilma Barcellini, Stefan W. Eber, Bertil Glader, Hassan M. Yaish, Satheesh Chonat, Mukta Sharma, Kevin H M Kuo, Ellis J. Neufeld, Heng Wang, Madeleine Verhovsek, Sujit Sheth, Rachael F. Grace

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: Pyruvate kinase (PK) deficiency is caused by PKLR gene mutations, leading to defective red blood cell glycolysis and hemolytic anemia. Rates of comorbidities and complications by transfusion history and relative to the general population remain poorly quantified.

METHODS: Data for patients aged ≥ 18 years with two confirmed PKLR mutations were obtained from the PK deficiency Natural History Study (NCT02053480). Frequencies of select conditions were compared with an age- and sex-matched cohort from a general insured US population without PK deficiency.

RESULTS: Compared with the matched population (n = 1220), patients with PK deficiency (n = 122) had significantly …

Severe Hyponatremia Following Cystoscopy And Urethral Dilation: An Unusual Late Complication, Mir Ibrahim Sajid, Shanila Ahmed, Noman Ali

Medical College Documents

No abstract provided.

Warm Autoimmune Hemolytic Anemia Associated With Asymptomatic Sars-Cov-2 Infection., Joseph R Liput, Kim Jordan, Rini Patadia, Elizabeth Kander

Ambulatory and Primary Care Articles

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and the resultant coronavirus disease 2019 (COVID-19) are associated with several hematologic abnormalities, including immune thrombocytopenia, antiphospholipid syndrome, and autoimmune hemolytic anemia (AIHA). Initial case reports suggested immune dysregulation to be the underlying etiology of SARS-CoV-2-associated AIHA, as all reported cases involved patients with moderate to severe COVID-19, many of whom had underlying lymphoproliferative disorders. More recently, AIHA has been reported in patients with mildly symptomatic SARS-CoV-2 infection. Here, we detail a patient with asymptomatic SARS-CoV-2 infection who presented with severe, symptomatic anemia. Workup was consistent with warm autoimmune hemolytic anemia (WAIHA) secondary …