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339 full-text articles. Page 6 of 14.

Total Versus Partial Splenectomy In Pediatric Hereditary Spherocytosis: A Systematic Review And Meta-Analysis, Leonardo Guizzetti 2016 Western University

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 ...


Discovery And Development Of Human Monoclonal Antibodies To Block Rhd Alloimmunization During Pregnancy, Tushar Gupta, Melissa Gawron, Colby A. Souders, Michael A. Brehm, Dale Greiner, Leonard D. Shultz, Smita Jaiswal, Sean McCauley, Ann Dauphin, Jeremy Luban, Lisa Cavacini 2016 University of Massachusetts Medical School

Discovery And Development Of Human Monoclonal Antibodies To Block Rhd Alloimmunization During Pregnancy, Tushar Gupta, Melissa Gawron, Colby A. Souders, Michael A. Brehm, Dale Greiner, Leonard D. Shultz, Smita Jaiswal, Sean Mccauley, Ann Dauphin, Jeremy Luban, Lisa Cavacini

UMass Center for Clinical and Translational Science Research Retreat

Exposure of an Rh negative mother to red blood cells (RBCs) of an Rh positive fetus results in alloimmunization and development of anti-RhD antibodies. The anti-RhD antibodies cause hemolytic disease of the new born babies during subsequent pregnancies. Current prophylactic treatment involves polyclonal anti-RhD IgG purified from plasma of humans and is administered in approximately 20% of pregnancies. While the current prophylaxis is effective, it involves the use of human plasma and non-RhD specific antibodies, thus posing a risk of transmitting infections and undesired antibody reactions. Moreover, there is a serious scarcity of plasma donors to meet the requirement of ...


The Mysteries Of Your Blood, Alexandra J. Mauer 2016 Butler University

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 2016 University of Oxford

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 2016 University of Nebraska Medical Center

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% of ...


Shortened Lifespan And Lethal Hemorrhage In A Hemophilia A Mouse Model, Janice M. Staber, Molly J Pollpeter 2016 University of Iowa

Shortened Lifespan And Lethal Hemorrhage In A Hemophilia A Mouse Model, Janice M. Staber, Molly J Pollpeter

Stead Family Department of Pediatrics Publications

BACKGROUND: Hemophilia A animal models have helped advance our understanding of factor VIII deficiency. Previously, factor VIII deficient mouse models were reported to have a normal life span without spontaneous bleeds. However, the bleeding frequency and survival in these animals has not been thoroughly evaluated.

OBJECTIVE: To investigate the survival and lethal bleeding frequency in two strains of E-16 hemophilia A mice.

METHODS: We prospectively studied factor VIII deficient hemizygous affected males (n = 83) and homozygous affected females (n = 55) for survival and bleeding frequency. Animals were evaluated for presence and location of bleeds as potential cause of death.

RESULTS ...


Cll Metabolism Is Regulated By Prognostic Factors, Modulated By Stroma And Abrogated By Pi3k Inhibition, Hima Vangapandu 2016 The University of Texas Graduate School of Biomedical Sciences at Houston

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

UT GSBS 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 ...


To What Extent Did Blood Transfusion Systems And Technologies Modernize During World War Ii?, Hannah J. LeBlanc 2016 Sunset High School

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 2016 Melaka-Manipal Medical College

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 2016 New York Medical College

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 ...


Ca19-9 Decrease At 8 Weeks As A Predictor Of Overall Survival In A Randomized Phase Iii Trial (Mpact) Of Weekly Nab-Paclitaxel Plus Gemcitabine Versus Gemcitabine Alone In Patients With Metastatic Pancreatic Cancer, Gabriela E. Chiorean, Venu G. Bathini 2016 University of Washington

Ca19-9 Decrease At 8 Weeks As A Predictor Of Overall Survival In A Randomized Phase Iii Trial (Mpact) Of Weekly Nab-Paclitaxel Plus Gemcitabine Versus Gemcitabine Alone In Patients With Metastatic Pancreatic Cancer, Gabriela E. Chiorean, Venu G. Bathini

Open Access Articles

BACKGROUND: A phase I/II study and subsequent phase III study (MPACT) reported significant correlations between CA19-9 decreases and prolonged overall survival (OS) with nab-paclitaxel plus gemcitabine (nab-P + Gem) treatment for metastatic pancreatic cancer (MPC). CA19-9 changes at week 8 and potential associations with efficacy were investigated as part of an exploratory analysis in the MPACT trial.

PATIENTS AND METHODS: Untreated patients with MPC (N = 861) received nab-P + Gem or Gem alone. CA19-9 was evaluated at baseline and every 8 weeks.

RESULTS: Patients with baseline and week-8 CA19-9 measurements were analyzed (nab-P + Gem: 252; Gem: 202). In an analysis pooling ...


A Case Report Of Ruxolitinib Induced Hypocalcemia: A Stochastic Or Deterministic Effect?, Arsha Sreedhar MD, Ranjit R. Nair MD, Gretchen A. Perilli MD 2016 Lehigh Valley Health Network

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 ...


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 2016 University of Texas at Dallas

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 ...


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 2016 Division of Clinical Epidemiology and Aging Research, German Cancer Research Center (DKFZ); Cardeza Foundation, Division of Hematology, Department of Medicine, Thomas Jefferson University

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 ...


Principles Of Medical Oncology, James Liebmann, Richard S. Pieters 2016 University of Massachusetts Medical School

Principles Of Medical Oncology, James Liebmann, Richard S. Pieters

Cancer Concepts: A Guidebook for the Non-Oncologist

Medical oncologists are internists specializing in the care of the adult cancer patient. They frequently function as a primary care physician for patients with cancer. This chapter in Cancer Concepts: A Guidebook for the Non-Oncologist will review the practice of medical oncology and some general principles followed in the use of chemotherapy. Concepts including goals of chemotherapy treatment, evaluation of the effects of chemotherapy, integration of chemotherapy with other cancer treatments, and use of combinations of chemotherapy drugs will be covered.


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 2016 Children's Mercy Hospital

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 ...


Conjugate Haemophilus Influenzae Type B Vaccines For Sickle Cell Disease., Slimane Allali, Martin Chalumeau, Odile Launay, Samir K. Ballas, Mariane de Montalembert 2016 Necker Hospital for Sick Children

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 2016 George Washington University

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 ...


Safety And Efficacy Of Blood Exchange Transfusion For Priapism Complicating Sickle Cell Disease., Samir K. Ballas, David Lyon 2016 Thomas Jefferson University

Safety And Efficacy Of Blood Exchange Transfusion For Priapism Complicating Sickle Cell Disease., Samir K. Ballas, David Lyon

Cardeza Foundation for Hematologic Research

BACKGROUND: Priapism is unwanted painful penile erection that affects about 36% of boys and men with sickle cell disease (SCD) most of whom have sickle cell anemia. Clinically, priapism could be stuttering, minor, or major. The first two types are mild, last < 4 h, are usually treated at home, have good prognosis with normal sexual function. The major type of priapism lasts >4 h, associated with severe pain, requires hospitalization; often does not respond to medical treatment and may require shunt surgery. Untreated major priapism and surgical intervention often cause impotence. In this study, we report our 15-year experience in treating adult patients with SCD and major priapism with blood exchange transfusion after being refractory to other medical therapies.

METHODS ...


Novel Oral Transforming Growth Factor-Beta Signaling Inhibitor Ew-7197 Eradicates Cml-Initiating Cells, Kazuhito Naka, Shaoguang Li, Dae-Kee Kim 2016 Kanazawa University

Novel Oral Transforming Growth Factor-Beta Signaling Inhibitor Ew-7197 Eradicates Cml-Initiating Cells, Kazuhito Naka, Shaoguang Li, Dae-Kee Kim

Open Access Articles

Recent strategies for treating CML patients have focused on investigating new combinations of tyrosine kinase inhibitors (TKIs) as well as identifying novel translational research agents that can eradicate CML leukemia-initiating cells (CML-LICs). However, little is known about the therapeutic benefits such CML-LIC targeting therapies might bring to CML patients. In this study, we investigated the therapeutic potential of EW-7197, an orally bioavailable transforming growth factor-beta signaling inhibitor which has recently been approved as an Investigational New Drug (NIH, USA), to suppress CML-LICs in vivo. Compared to TKI treatment alone, administration of TKI plus EW-7197 to CML-affected mice significantly delayed disease ...


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