Hematology Commons^™

The Hemostatic Effects Of Acute Exposure To Colored Cornstarch Powder During A 5k Run, Robert C. Allsbrook

Senior Honors Projects, 2010-2019

PURPOSE: To examine the acute hemostatic effects of particulate matter (PM) in the form of colored corn starch powder during a 5 kilometer race. METHODS: 10 recreationally active adults completed two 5k runs, one with color and one without color. 10 mL blood samples were taken 6 hours prior to the trial and immediately following the trial. PAI-1 activity, FVIII antigen, and tPA activity were measured using an ELISA. RESULTS: No significant main effects or interaction effects (P<0.05) were observed among any of the variables although a trend (P = 0.082) was observed for increased PAI-1 activity during exercise in the color condition. CONCLUSIONS: There is a trend towards increased PAI-1 activity levels during exercise during color runs that could be due to increased inflammation. Furthermore, the lack of increased FVIII:ag and tPA activity suggests that the intensity of the exercise may not have been adequate and further research should be conducted in this area.

Stars In Cytoplasm: Pediatric Neuroblastoma, Monazza Chaudhary, Shahzad Sarwar, Natasha Bahadur Ali

Section of Haematology/Oncology

We report a case of a 2-year-old female child who presented with abdominal distention and fever. On examination, there was a palpable mass in the right hypochondrium. Computed tomography of the abdomen revealed a large mass in the right suprarenal area. Biopsy of the mass revealed malignant round blue cell neoplasm. Bone marrow examination showed diffuse infiltration with atypical mononuclear cells and cytogenetic studies showed positivity for NMyc translocation.

A Trial Of Unrelated Donor Marrow Transplantation For Children With Severe Sickle Cell Disease., Shalini Shenoy, Mary Eapen, Julie A. Panepinto, Brent R. Logan, Juan Wu, Allistair Abraham, Joel Brochstein, Sonali Chaudhury, Kamar Godder, Ann E. Haight, Kimberly A. Kasow, Kathryn Leung, Martin Andreansky, Monica Bhatia, Jignesh Dalal, Hilary Haines, Jennifer Jaroscak, Hillard M. Lazarus, John E. Levine, Lakshmanan Krishnamurti, David Margolis, Gail C. Megason, Lolie C. Yu, Michael A. Pulsipher, Iris Gersten, Nancy Difronzo, Mary M. Horowitz, Mark C. Walters, Naynesh Kamani

Manuscripts, Articles, Book Chapters and Other Papers

Children with sickle cell disease experience organ damage, impaired quality of life, and premature mortality. Allogeneic bone marrow transplant from an HLA-matched sibling can halt disease progression but is limited by donor availability. A Blood and Marrow Transplant Clinical Trials Network (BMT CTN) phase 2 trial conducted from 2008 to 2014 enrolled 30 children aged 4 to 19 years; 29 were eligible for evaluation. The primary objective was 1-year event-free survival (EFS) after HLA allele-matched (at HLA-A, -B, -C, and -DRB1 loci) unrelated donor transplant. The conditioning regimen included alemtuzumab, fludarabine, and melphalan. Graft-versus-host disease (GVHD) prophylaxis included calcineurin inhibitor, …

Splenic Infarction: An Uncommon Presentation Of Acute Infectious Mononucleosis, Madhulika Urella, Roma Srivastava, Waseem Ahmed, Yehuda Lebowicz

Marshall Journal of Medicine

Splenic infarction is a relatively uncommon diagnosis. It occurs when the splenic artery or one of its sub-branches is occluded with an infected or bland embolus or clot. Splenic infarction may be caused by atrial fibrillation, bacterial endocarditis, sickle cell disease, antiphospholipid syndrome, and trauma whereas an infectious etiology is uncommon. It is considered a rare presentation of acute infectious mononucleosis. Currently, its pathogenesis is still unclear. We describe a 24-year-old African American female who was admitted for evaluation of left-sided chest pain. Chest imaging, abdominal ultrasound, and initial laboratory data were normal, followed by a negative hypercoagulability panel. Signs …

Primary Malignant Pleural Effusion With A Profound Type B Lactic Acidosis, Rachel Kinney Do, Christine Saraceni Do, Eliot L. Friedman Md

Department of Medicine

No abstract provided.

Feasibility Of A Community-Based Sickle Cell Trait Testing And Counseling Program, Ashley Housten, Regina Abel, Terianne Lindsey, Allison King

Journal of Health Disparities Research and Practice

Background: Sickle cell trait (SCT) screening is required at birth in the United States; however, adults rarely know their SCT status prior to having children.

Purpose: Assess feasibility of a community-based SCT education and testing intervention.

Methods: Participants were recruited from eight community sites to complete an educational program and offered a hemoglobin analysis. A genetic counselor met individually with participants to discuss lab results.

Results: Between July 14, 2010 and May 31, 2012, 637 participants completed the educational program. Five hundred seventy (89.5%) provided a blood sample, and 61 (10.9%) had SCT or other hemoglobinopathies. The genetic counselor met …

Hemophagocytic Lymphohistiocytosis, An Overlooked Culprit Of Disseminated Intravascular Coagulation, Shivtaj Mann Do

Department of Medicine

No abstract provided.

Prevalence Of Stroke In Children Admitted With Sickle Cell Anaemia To Mulago Hospital, Samden Lhatoo, Martha Sajatovic

Faculty Scholarship

Background: Stroke is a major complication of sickle cell anaemia (SCA). It occurs commonly in childhood with about 10 % of children with sickle cell anaemia getting affected by this complication. In Uganda, there is paucity of data on the prevalence of stroke in children admitted in a tertiary institution. We determined the prevalence of stroke amongst children with SCA admitted to Mulago National Referral Hospital in Uganda and described the ir co-morbidities. Methods: We conducted a retrospective record review of children with SCA admitted from August 2012 to August 2014 to the Paediatric Haematology Ward of Mulago Hospital in …

A Predictive Model For Intensive Chemotherapy Outcomes In Newly Diagnosed Elderly Patients With Aml, Sameer Tolay Md, David F. Claxton Md, Junjia Zhu Md

Department of Medicine

No abstract provided.

Adrenal Incidentaloma In Young – A Gray Zone?, Ranjit R. Nair Md, Stacey Smith Md, Facp

Department of Medicine

No abstract provided.

Peritoneal Lymphomatosis: The Failure Of Occam’S Razor, Ranjit R. Nair Md, Nicole M. Agostino Do, Shereen M F Gheith Md, Usman Shah Md

Department of Medicine

No abstract provided.

Mutations Affecting Epigenetic Regulators And Their Role In Peripheral T-Cell Lymphomas, Chao Wang

Theses & Dissertations

Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of generally aggressive lymphoid malignancies, accounting for 10-15% of all non-Hodgkin lymphomas. Angioimmunoblastic T-cell lymphoma (AITL) represents approximately 20% of all PTCLs and is recognized as a distinct entity. Accurate diagnosis and classification of PTCL remain challenging. With the exception of ALK+ ALCL, patients with PTCL generally have a poor prognosis with standard chemotherapy and even with the availability of many novel drugs, including HDAC inhibitor (romidepsin and belinostat), gemcitabine, and bortezomib. Therefore, deciphering the pathogenesis of this group of diseases is needed to identify novel treatable targets for better therapeutic intervention. …

Pattern Of Relapse In Paediatric Acute Lymphoblastic Leukaemia In A Tertiary Care Unit, Emaduddin Siddiqui, Sayyeda Ghazala Kazi, Muhammad Irfan Habib, Khalid Mehmood Ahmed Khan, Nukhba Zia

Department of Emergency Medicine

OBJECTIVE:

To determine the frequency, site and time to relapse from diagnosis, and to see the relationship of relapse with important prognostic factors.

METHODS:

The prospective descriptive observational study was conducted at the National Institute of Child Health, Karachi, June 2005 to May 2007, and comprised newly-diagnosed cases of acute lymphoblastic leukaemia. Bone marrow aspiration was done on reappearance of blast cells in peripheral smear and cerebrospinal fluid. Detailed report was done each time when intra-thecal chemotherapy was given or there were signs and symptoms suggestive of central nervous system relapse. SPSS 12 was used for data analysis.

RESULTS:

Of …

Immunoglobulin G4-Related Disease: A Rare Disease With An Unusual Presentation, Muhammad Waqas Khan, Terrance Hadley, Melissa Kesler, Zartash Gul

Internal Medicine Faculty Publications

IgG4-RD can also present in the skeletal muscle, mimicking several other diseases. It is unusual for this relatively new classification of diseases to present in the muscles and can be mistakenly diagnosed as other autoimmune diseases rendering a delay in the appropriate management and progression of the disease.

Age- And Sex-Related Changes In Fasting Plasma Glucose And Lipoprotein In Cynomolgus Monkeys, Feng Yue, Guodong Zhang, Rongping Tang, Zhouquan Zhang, Liqiong Teng, Zhiming Zhang

Neuroscience Faculty Publications

Background: The age-related dysfunction of glucose and lipid metabolism has a long-standing relationship with cardiovascular and neurodegenerative disease. However, the effects of metabolic dysfunction on men and women are different. Reasons for these sex differences remains unclear. Cynomolgus monkeys have been used, in the past, for the study of human metabolic diseases due to their biologically proximity to humans. Nevertheless, few studies to date have focused on both age- and sex-related differences in glucose and lipid metabolism. The present study was designed to specifically address these questions by using a large cohort of cynomolgus monkeys (N = 1,399) including …

Total Versus Partial Splenectomy In Pediatric Hereditary Spherocytosis: A Systematic Review And Meta-Analysis, Leonardo Guizzetti

Epidemiology and Biostatistics Publications

To compare the clinical effectiveness of total (TS) or partial (PS) splenectomy in pediatric hereditary spherocytosis, a systematic review and meta-analysis was performed (PROSPERO registration CRD42015030056). There were 14 observational studies comparing pre- and post- operative hematologic parameters. Secondary outcomes include in-hospital infections, surgical complications, symptomatic recurrence and biliary disease. TS is more effective than PS to increase hemoglobin (3.6 g/dL vs 2.2 g/dL) and reduce reticulocytes (12.5% vs 6.5%) after one year; outcomes following PS are stable for at least 6 years. There were no cases of overwhelming post-splenectomy sepsis. A population-based patient registry is needed for long-term follow-up.

The Mysteries Of Your Blood, Alexandra J. Mauer

BU Well

Did you know that your blood type can be useful for determining disease susceptibility and even creating a diet that works best for your personal metabolism? The Mysteries of your Blood is an article that delves into the science behind the ABO blood types and explains why your body can handle certain foods, stress, and illness differently than someone with a different blood type. It proposes a new, perplexing way to determine how to avoid certain health problems while at the same time offering suggestions of what to eat or, rather, what to avoid eating.

Pharmacological Interventions For Painful Sickle Cell Vaso-Occlusive Crises In Adults, Tess E. Cooper, Ian R. Hambleton, Samir K. Ballas, Philip J. Wiffen

Cardeza Foundation for Hematologic Research

This is the protocol for a review and there is no abstract. The objectives are as follows: To assess the analgesic efficacy, and adverse events, of pharmacological interventions used to treat acute painful sickle cell vaso-occlusive crises in adults, aged 18 and over, in any setting.

Recurrent Mutations Of T-Cell Receptor And Co-Stimulatory Signaling Proteins In Peripheral T-Cell Lymphomas, Joseph Rohr

Theses & Dissertations

Peripheral T-cell lymphomas (PTCLs) comprise a heterogeneous group of mature T-cell neoplasms with a poor prognosis. Recently, mutations in TET2 and other epigenetic modifiers as well as RHOA have been identified in these diseases, particularly in angioimmunoblastic T-cell lymphoma (AITL). CD28 is the major co-stimulatory receptor in T-cells which, upon binding ligand, induces sustained T-cell proliferation and cytokine production when combined with T-cell receptor stimulation, through many signaling molecules including VAV1. This thesis identifies recurrent mutations in CD28 in PTCLs, as well as mutations in VAV1. Two residues of CD28 – D124 and T195 – were recurrently mutated in 11.3% …

Comparing Direct Factor Xa Inhibitors And Warfarin In The Prevention Of Stroke In Patients With Atrial Fibrillation, Alessandra Lof, Stephanie Pillai

Physician Assistant Capstones, 2016 to 2019

Objective: To evaluate the overall efficacy, advantages, and disadvantages of treatment with direct factor Xa inhibitors as compared to warfarin in the prevention of stroke in patients with atrial fibrillation. Methods: A quantitative meta-analysis was performed on three separate studies, each of which evaluated the efficacy and safety outcomes of a direct factor Xa inhibitor versus warfarin in preventing stroke in patients with atrial fibrillation. The direct factor Xa inhibitors that were evaluated included apixaban, edoxaban, and rivaroxaban. Results: The direct factor Xa inhibitors were found to be as effective, and in some cases more effective, than …

Cll Metabolism Is Regulated By Prognostic Factors, Modulated By Stroma And Abrogated By Pi3k Inhibition, Hima Vangapandu

Dissertations and Theses (Open Access)

Metabolism of chronic lymphocytic leukemia (CLL), a disease characterized by the relentless accumulation of mature B cells has been little explored. Bone marrow stromal cells provide a survival benefit to CLL cells, in part through PI3K/AKT pathway. Compared with proliferative B-cell lines, metabolic fluxes of oxygen and lactate were low in quiescent malignant B lymphocytes from CLL patients. Glycolysis (extracellular acidification rate, ECAR) was consistently low in CLL samples, but oxygen consumption (OCR) varied considerably. Higher OCR was associated with poor prognostic factors such as ZAP 70 positivity, unmutated IgVH, high β2M levels, and higher Rai stage. Co-culture with the …

To What Extent Did Blood Transfusion Systems And Technologies Modernize During World War Ii?, Hannah J. Leblanc

Young Historians Conference

This investigation will explore changes in blood transfusion during World War II (1939-1945). Medical technology and collection systems for whole blood and blood plasma in particular will be examined. The focus of this investigation will be the United States, but Great Britain will also be mentioned due to its close blood transfusion-related interactions with the United States during this period. Additionally, blood transfusion prior to World War II and in modern times will also be considered in this investigation to provide context and to allow assessment of modernization during the war. However, artificial blood substitutes will not be considered, nor …

Treatment Of Dental Complications In Sickle Cell Disease., Priti Mulimani, Samir K. Ballas, Adinegara B L Abas, Laxminarayan Karanth

Cardeza Foundation for Hematologic Research

BACKGROUND: Sickle cell disease is the most common single gene disorder and the commonest haemoglobinopathy found with high prevalence in many populations across the world. Management of dental complications in people with sickle cell disease requires special consideration for three main reasons. Firstly, dental and oral tissues are affected by the blood disorder resulting in several oro-facial abnormalities. Secondly, living with a haemoglobinopathy and coping with its associated serious consequences may result in individuals neglecting their oral health care. Finally, the treatment of these oral complications must be adapted to the systemic condition and special needs of these individuals, in …

Clinical Factors Associated With Long-Term Complete Remission Versus Poor Response To Chemotherapy In Hiv-Infected Children And Adolescents With Kaposi Sarcoma Receiving Bleomycin And Vincristine: A Retrospective Observational Study, Nader K. El-Mallawany, William Kamiyango, Jeremy Kim Slone, Jimmy Villiera, Carrie L. Kovarik, Carrie M. Cox, Dirk Dittmer, Saeed Ahmed, Gordon E. Schutze, Michael E. Scheurer, Peter N. Kazembe, Parth S. Mehta

NYMC Faculty Publications

Kaposi sarcoma (KS) is the most common HIV-associated malignancy in children and adolescents in Africa. Pediatric KS is distinct from adult disease. We evaluated the clinical characteristics associated with long-term outcomes. We performed a retrospective observational analysis of 70 HIV-infected children and adolescents with KS less than 18 years of age diagnosed between 8/2010 and 6/2013 in Lilongwe, Malawi. Local first-line treatment included bleomycin and vincristine plus nevirapine-based highly active anti-retroviral therapy (HAART). Median age was 8.6 years (range 1.7-17.9); there were 35 females (50%). Most common sites of presentation were: lymph node (74%), skin (59%), subcutaneous nodules (33%), oral …

A Case Report Of Ruxolitinib Induced Hypocalcemia: A Stochastic Or Deterministic Effect?, Arsha Sreedhar Md, Ranjit R. Nair Md, Gretchen A. Perilli Md

Department of Medicine

Ruxolitinib is a novel selective JAK 1/2 inhibitor approved for the treatment of myelofibrosis (MF) and polycythemia vera (PCV). Hypocalcemia associated with ruxolitinib has not been reported in early trials or in literature.

A 65 year female with history of CKD stage 3, PCV since 1989, papillary thyroid carcinoma, total thyroidectomy and hypoparathyroidism since 1996 presented with complaints of severe myalgia, fatigue, paresthesia and critical hypocalcemia. Her corrected S. Ca 5.8 mg /dl (8.9-10.1 mg/dL) and Ionised Ca 2.9 mg/dl (4.5 - 5.4 mg/dL). Other labs showed stable creatinine 1.5 mg/dL, intact PTH 29 pg/ml(14-72pg/ml) and 25,OH vitamin D level …

A Case-Control Genome-Wide Association Study Identifies Genetic Modifiers Of Fetal Hemoglobin In Sickle Cell Disease., Li Liu, Alexander Pertsemlidis, Liang-Hao Ding, Michael D Story, Martin H Steinberg, Paola Sebastiani, Carolyn Hoppe, Samir K. Ballas, Betty S Pace

Cardeza Foundation for Hematologic Research

Sickle cell disease (SCD) is a group of inherited blood disorders that have in common a mutation in the sixth codon of the β-globin (HBB) gene on chromosome 11. However, people with the same genetic mutation display a wide range of clinical phenotypes. Fetal hemoglobin (HbF) expression is an important genetic modifier of SCD complications leading to milder symptoms and improved long-term survival. Therefore, we performed a genome-wide association study (GWAS) using a case-control experimental design in 244 African Americans with SCD to discover genetic factors associated with HbF expression. The case group consisted of subjects with HbF≥8.6% (133 samples) …

Trends In Survival Of Chronic Lymphocytic Leukemia Patients In Germany And The Usa In The First Decade Of The Twenty-First Century., Dianne Pulte, Felipe A Castro, Lina Jansen, Sabine Luttmann, Bernd Holleczek, Alice Nennecke, Meike Ressing, Alexander Katalinic, Hermann Brenner

Cardeza Foundation for Hematologic Research

BACKGROUND: Recent population-based studies in the United States of America (USA) and other countries have shown improvements in survival for patients with chronic lymphocytic leukemia (CLL) diagnosed in the early twenty-first century. Here, we examine the survival for patients diagnosed with CLL in Germany in 1997-2011.

METHODS: Data were extracted from 12 cancer registries in Germany and compared to the data from the USA. Period analysis was used to estimate 5- and 10-year relative survival (RS).

RESULTS: Five- and 10-year RS estimates in 2009-2011 of 80.2 and 59.5%, respectively, in Germany and 82.4 and 64.7%, respectively, in the USA were …

Pharmacokinetics And Bioequivalence Of A Liquid Formulation Of Hydroxyurea In Children With Sickle Cell Anemia., Jeremie H. Estepp, Chiara Melloni, Courtney D. Thornburg, Paweł Wiczling, Zora Rogers, Jennifer A. Rothman, Nancy S. Green, Robert Liem, Amanda M. Brandow, Shelley E. Crary, Thomas H. Howard, Maurine H. Morris, Andrew Lewandowski, Uttam Garg, William J. Jusko, Kathleen A. Neville, Best Pharmaceuticals For Children Act-Pediatric Trials Network Administrative Core Committee

Manuscripts, Articles, Book Chapters and Other Papers

Hydroxyurea (HU) is a crucial therapy for children with sickle cell anemia, but its off-label use is a barrier to widespread acceptance. We found HU exposure is not significantly altered by liquid vs capsule formulation, and weight-based dosing schemes provide consistent exposure. HU is recommended for all children starting as young as 9 months of age with sickle cell anemia (SCA; HbSS and HbSβspan(0) thalassemia); however; a paucity of pediatric data exists regarding the pharmacokinetics (PK) or the exposure-response relationship of HU. This trial aimed to characterize the PK of HU in children and to evaluate and compare the bioavailability …

Conjugate Haemophilus Influenzae Type B Vaccines For Sickle Cell Disease., Slimane Allali, Martin Chalumeau, Odile Launay, Samir K. Ballas, Mariane De Montalembert

Cardeza Foundation for Hematologic Research

BACKGROUND: People affected with sickle cell disease are at high risk of infection from Haemophilus influenzae type b. Before the implementation of Haemophilus influenzae type b conjugate vaccination in high-income countries, this was responsible for a high mortality rate in children under five years of age. In African countries, where coverage of this vaccination is still extremely low, Haemophilus influenzae type b remains one of the most common cause of bacteraemias in children with sickle cell disease. The increased uptake of this conjugate vaccination may substantially improve the survival of children with sickle cell disease.

OBJECTIVES: The primary objective was …

Hydroxycarbamide Versus Chronic Transfusion For Maintenance Of Transcranial Doppler Flow Velocities In Children With Sickle Cell Anaemia-Tcd With Transfusions Changing To Hydroxyurea (Twitch): A Multicentre, Open-Label, Phase 3, Non-Inferiority Trial., Russell E Ware, Barry R Davis, William H Schultz, R Clark Brown, Banu Aygun, Sharada Sarnaik, Lori Luchtman-Jones, +Several Additional Authors

Pediatrics Faculty Publications

BACKGROUND: For children with sickle cell anaemia and high transcranial doppler (TCD) flow velocities, regular blood transfusions can effectively prevent primary stroke, but must be continued indefinitely. The efficacy of hydroxycarbamide (hydroxyurea) in this setting is unknown; we performed the TWiTCH trial to compare hydroxyurea with standard transfusions.

METHODS: TWiTCH was a multicentre, phase 3, randomised, open-label, non-inferiority trial done at 26 paediatric hospitals and health centres in the USA and Canada. We enrolled children with sickle cell anaemia who were aged 4-16 years and had abnormal TCD flow velocities (≥ 200 cm/s) but no severe vasculopathy. After screening, eligible …