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Hemic and Lymphatic Diseases Commons

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Investigation Of A Peculiar Case Of Childhood Lymphadenopathy, Chade Aribo, Alonso Al, Allison Woodall, Tommy Y. Kim 2020 University of California Riverside

Investigation Of A Peculiar Case Of Childhood Lymphadenopathy, Chade Aribo, Alonso Al, Allison Woodall, Tommy Y. Kim

HCA Healthcare Journal of Medicine

Introduction: Lymphadenopathy is a common physical finding often associated with an infectious etiology. We present a case of a patient with generalized cervical and supraclavicular lymphadenopathy diagnosed with a rare malignancy. Health care providers should be aware of some uncommon historical or physical examination findings that warrant further investigation.

Clinical Findings: A 16-year-old female presented to the emergency department with 3 days of a fever, congestion and sore throat with swelling around the jaw. Her physical examination findings included bilateral mandibular swelling and generalized cervical lymphadenopathy with palpable supraclavicular lymph nodes.

Outcomes: Complete blood count showed pancytopenia with a white ...


Reduced Intensity Conditioning For Acute Myeloid Leukemia Using Melphalan- Vs Busulfan-Based Regimens: A Cibmtr Report, Zheng Zhou, Jan Cerny 2020 Lahey Hospital and Medical Center

Reduced Intensity Conditioning For Acute Myeloid Leukemia Using Melphalan- Vs Busulfan-Based Regimens: A Cibmtr Report, Zheng Zhou, Jan Cerny

Open Access Articles

There is a lack of large comparative study on the outcomes of reduced intensity conditioning (RIC) in acute myeloid leukemia (AML) transplantation using fludarabine/busulfan (FB) and fludarabine/melphalan (FM) regimens. Adult AML patients from Center for International Blood and Marrow Transplant Research who received first RIC allo-transplant between 2001 and 2015 were studied. Patients were excluded if they received cord blood or identical twin transplant, total body irradiation in conditioning, or graft-versus-host disease (GVHD) prophylaxis with in vitro T-cell depletion. Primary outcome was overall survival (OS), secondary end points were leukemia-free survival (LFS), nonrelapse mortality (NRM), relapse, and GVHD ...


Medical Resource Use And Costs Of Treating Sickle Cell-Related Vaso-Occlusive Crisis Episodes: A Retrospective Claims Study, Nirmish Shah, Menaka Bhor, Lin Xie, Jincy Paulose, Huseyin Yuce 2020 Duke University

Medical Resource Use And Costs Of Treating Sickle Cell-Related Vaso-Occlusive Crisis Episodes: A Retrospective Claims Study, Nirmish Shah, Menaka Bhor, Lin Xie, Jincy Paulose, Huseyin Yuce

Publications and Research

Background: The study investigated the economic burden of vaso-occlusive crisis (VOC) among sickle cell disease (SCD) patients, through assessment of overall utilization and costs and costs per VOC episode (regarding the number of VOC episodes and health care setting, respectively).

Methods: Using the Medicaid Analytic Extracts database, the first SCD-related diagnosis claim (index claim) between June 1, 2009–December 31, 2012 was identified among eligible adults. Patients were required to have continuous medical and pharmacy benefits for 6 months pre- and 12 months post-index. Discrete VOC claims identified within a 3-day gap were combined as a single VOC episode. Annual ...


Effectiveness Of Aerobic Exercise For Adults With Leukemia, Jalin Davis, Summer Hood, Alex Miller, Sam Stein, Kaylee Stem 2020 University of Tennessee Health Science Center

Effectiveness Of Aerobic Exercise For Adults With Leukemia, Jalin Davis, Summer Hood, Alex Miller, Sam Stein, Kaylee Stem

Master of Occupational Therapy Student Critically Appraised Topics

We wanted to look at the most effective interventions for increasing strength and endurance in clients with Leukemia experiencing functional decline.


Survival Following Allogeneic Transplant In Patients With Myelofibrosis, Krisstina Gowin, Jan Cerny 2020 University of Arizona

Survival Following Allogeneic Transplant In Patients With Myelofibrosis, Krisstina Gowin, Jan Cerny

Open Access Articles

Allogeneic hematopoietic cell transplantation (HCT) is the only curative therapy for myelofibrosis (MF). In this large multicenter retrospective study, overall survival (OS) in MF patients treated with allogeneic HCT (551 patients) and without HCT (non-HCT) (1377 patients) was analyzed with Cox proportional hazards model. Survival analysis stratified by the Dynamic International Prognostic Scoring System (DIPSS) revealed that the first year of treatment arm assignment, due to upfront risk of transplant-related mortality (TRM), HCT was associated with inferior OS compared with non-HCT (non-HCT vs HCT: DIPSS intermediate 1 [Int-1]: hazard ratio [HR] = 0.26, P < .0001; DIPSS-Int-2 and higher: HR, 0.39, P < .0001). Similarly, in the DIPSS low-risk MF group, due to upfront TRM risk, OS was superior with non-HCT therapies compared with HCT in the first-year post treatment arm assignment (HR, 0.16, P = .006). However, after 1 year, OS was not significantly different (HR, 1.38, P = .451). Beyond 1 year of treatment arm assignment, an OS advantage with HCT therapy in Int-1 and higher DIPSS score patients was observed (non-HCT vs HCT: DIPSS-Int-1: HR, 2.64, P < .0001; DIPSS-Int-2 and higher: HR, 2.55, P < .0001). In conclusion, long-term OS advantage with HCT was observed for patients with Int-1 or higher risk MF, but at the cost of early TRM. The magnitude of OS benefit with HCT increased as DIPSS risk score increased and became apparent with longer follow-up.


A Unique Presentation Of Cutaneous Diffuse Large B-Cell Lymphoma, Mary Awad, Erik Holzwanger, Sandeep Jubbal 2020 University of Massachusetts Medical School

A Unique Presentation Of Cutaneous Diffuse Large B-Cell Lymphoma, Mary Awad, Erik Holzwanger, Sandeep Jubbal

Open Access Articles

Cutaneous B-cell lymphomas (CBCL) are rare heterogeneous neoplastic diseases composing about 22.5% of all cutaneous lymphomas. These diseases can be divided into primary and secondary cutaneous variants with primary cutaneous B-cell lymphoma (PCBCL) divided into three distinct entities including primary cutaneous marginal zone lymphoma, primary cutaneous follicle center lymphoma, and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT). Secondary cutaneous diffuse large B-cell lymphoma (CDLBCL) and PCDLBCL, LT are more aggressive neoplasms compared to the aforementioned CBCL with survival rates of 37% and 50% after 5 years, respectively. CDLBCL can present as cutaneous or subcutaneous nodules, papular ...


Epstein-Barr Virus Epitope-Major Histocompatibility Complex Interaction Combined With Convergent Recombination Drives Selection Of Diverse T Cell Receptor Alpha And Beta Repertoires, Anna Gil, Larisa Kamga, Ramakanth Chirravuri-Venkata, Nuray Aslan, Fransenio G. Clark, Dario Ghersi, Katherine Luzuriaga, Liisa K. Selin 2020 University of Massachusetts Medical School

Epstein-Barr Virus Epitope-Major Histocompatibility Complex Interaction Combined With Convergent Recombination Drives Selection Of Diverse T Cell Receptor Alpha And Beta Repertoires, Anna Gil, Larisa Kamga, Ramakanth Chirravuri-Venkata, Nuray Aslan, Fransenio G. Clark, Dario Ghersi, Katherine Luzuriaga, Liisa K. Selin

Open Access Articles

Recognition modes of individual T cell receptors (TCRs) are well studied, but factors driving the selection of TCR repertoires from primary through persistent human virus infections are less well understood. Using deep sequencing, we demonstrate a high degree of diversity of Epstein-Barr virus (EBV)-specific clonotypes in acute infectious mononucleosis (AIM). Only 9% of unique clonotypes detected in AIM persisted into convalescence; the majority (91%) of unique clonotypes detected in AIM were not detected in convalescence and were seeming replaced by equally diverse "de novo" clonotypes. The persistent clonotypes had a greater probability of being generated than nonpersistent clonotypes due ...


Isolated Forefoot Juvenile Xanthogranuloma: Unique Case Study And Treatment In A Pediatric Patient, Brian Derner DPM, Kristine Hoffman, Amy Storfa MD, Dustin Kruse DPM, Paul A. Stone DPM, FACFAS 2020 HCA Healthcare

Isolated Forefoot Juvenile Xanthogranuloma: Unique Case Study And Treatment In A Pediatric Patient, Brian Derner Dpm, Kristine Hoffman, Amy Storfa Md, Dustin Kruse Dpm, Paul A. Stone Dpm, Facfas

Podiatry

Juvenile xanthogranulomas (JXG) are a benign histiocytic cell proliferative disorder in early childhood. They most commonly present within the first two years of life with papular or nodular changes to the skin on the head, neck or upper trunk. Xanthogranulomas are mostly self-limiting, can be singular or multiple, and rarely can infer a systemic disease process1. The purpose of this study was to publish a unique finding of an extra-tendinous solitary mass in an otherwise healthy 17-year old, with surgical and medical treatment after diagnosis.


Bradycardia In The Setting Of Gastric Sarcoidosis, Kameron Black MPH, Michelle Levine DO, Rage Geringer MD, Gregory Hicks MD, Oliwier Dziadkowiec 2020 HCA Healthcare

Bradycardia In The Setting Of Gastric Sarcoidosis, Kameron Black Mph, Michelle Levine Do, Rage Geringer Md, Gregory Hicks Md, Oliwier Dziadkowiec

Internal Medicine

No abstract provided.


Investigating The Nucleophosmin (Npm1) Protein In The Context Of Protein Aggregation In The Budding Yeast S. Cerevisiae, Tyler Atagozli 2020 University of Iowa

Investigating The Nucleophosmin (Npm1) Protein In The Context Of Protein Aggregation In The Budding Yeast S. Cerevisiae, Tyler Atagozli

Honors Theses at the University of Iowa

The Nucleophosmin (Npm1) protein is a nucleolar protein found in jawed vertebrates. It is primarily involved in ribosome biogenesis. Recent studies have proposed a new role for this protein as a protective agent colocalizing with cellular aggregates like firefly luciferase and the mutant huntingtin protein during stress conditions. In this study, the behavior of Npm1 was investigated in the budding yeast S. cerevisiae by fluorescence microscopy to see if Npm1 colocalizes or otherwise interacts with aggregates. I found that Npm1 redistributes to a subnuclear location after heat shock where it may colocalize with luciferase aggregates. HTTQ103, a mutant huntingtin protein ...


Curing Hemophilia A By Nhej-Mediated Ectopic F8 Insertion In The Mouse, Jian-Ping Zhang, Xin-Xin Cheng, Mei Zhao, Guo-Hua Li, Jing Xu, Feng Zhang, Meng-Di Yin, Fei-Ying Meng, Xin-Yue Dai, Ya-Wen Fu, Zhi-Xue Yang, Cameron Arakaki, Ruijun Jeanna. Su, Wei Wen, Wen-Tian Wang, Wanqiu Chen, Hannah Choi, Charles Wang, Guangping Gao, Lei Zhang, Tao Cheng, Xiao-Bing Zhang 2019 Peking Union Medical College

Curing Hemophilia A By Nhej-Mediated Ectopic F8 Insertion In The Mouse, Jian-Ping Zhang, Xin-Xin Cheng, Mei Zhao, Guo-Hua Li, Jing Xu, Feng Zhang, Meng-Di Yin, Fei-Ying Meng, Xin-Yue Dai, Ya-Wen Fu, Zhi-Xue Yang, Cameron Arakaki, Ruijun Jeanna. Su, Wei Wen, Wen-Tian Wang, Wanqiu Chen, Hannah Choi, Charles Wang, Guangping Gao, Lei Zhang, Tao Cheng, Xiao-Bing Zhang

Open Access Articles

BACKGROUND: Hemophilia A, a bleeding disorder resulting from F8 mutations, can only be cured by gene therapy. A promising strategy is CRISPR-Cas9-mediated precise insertion of F8 in hepatocytes at highly expressed gene loci, such as albumin (Alb). Unfortunately, the precise in vivo integration efficiency of a long insert is very low (~ 0.1%).

RESULTS: We report that the use of a double-cut donor leads to a 10- to 20-fold increase in liver editing efficiency, thereby completely reconstituting serum F8 activity in a mouse model of hemophilia A after hydrodynamic injection of Cas9-sgAlb and B domain-deleted (BDD) F8 donor plasmids. We ...


Outbreak Of Ebola Virus Disease, Alexandra Dimit, Tiffany Kneuss, Joelle Farano, Haley Armstrong, Jodi Otte, Andrew M. Roecker 2019 Ohio Northern University

Outbreak Of Ebola Virus Disease, Alexandra Dimit, Tiffany Kneuss, Joelle Farano, Haley Armstrong, Jodi Otte, Andrew M. Roecker

Pharmacy and Wellness Review

Ebola virus disease (EVD) has existed as a major health concern with devastating and, many times, fatal symptoms. The recent outbreaks of EVD in West Africa and the Democratic Republic of the Congo (DRC) have incited international concern. In this article, the implications of EVD will be discussed including the etiology, transmission, signs and symptoms, diagnosis and treatment of the disease. In addition to this discussion, the manner in which major health care organizations, including the World Health Organization (WHO), are dealing with treating infected patients and containing spread of the disease will be covered.


Laryngeal Mucous Membrane Plasmacytosis With 15 Year Follow-Up: Case Report And Literature Review, Vladislav V. Makarenko, Alec E. Vaezi, Doreen B. Brettler, Lloyd Hutchinson, Bruce A. Woda, Benjamin J. Chen 2019 University of Massachusetts Medical School

Laryngeal Mucous Membrane Plasmacytosis With 15 Year Follow-Up: Case Report And Literature Review, Vladislav V. Makarenko, Alec E. Vaezi, Doreen B. Brettler, Lloyd Hutchinson, Bruce A. Woda, Benjamin J. Chen

Open Access Articles

Mucous membrane plasmacytosis (MMP) is an uncommon variant of mucositis represented by a polyclonal plasma cell infiltration of mucosal tissue. Various clinical presentations in the upper airway have been reported ranging from erythematous mucosa to fungating masses. Histologic features include mucosal epithelial hyperplasia or psoriasiform changes with a dense submucosal infiltrate of polytypic plasma cells. Molecular studies for immunoglobulin gene rearrangement should be performed in all cases of MMP to rule out clonal neoplastic expansion of plasma cells. We present a case of MMP with over 15 years of clinical follow-up, emphasizing the relatively benign clinical course of this disorder.


Fanconi-Brca Pathway Mutations In Childhood T-Cell Acute Lymphoblastic Leukemia, Gayle P. Pouliot, Justine E. Roderick, Alejandro Gutierrez 2019 Boston Children's Hospital

Fanconi-Brca Pathway Mutations In Childhood T-Cell Acute Lymphoblastic Leukemia, Gayle P. Pouliot, Justine E. Roderick, Alejandro Gutierrez

Open Access Articles

BRCA2 (also known as FANCD1) is a core component of the Fanconi pathway and suppresses transformation of immature T-cells in mice. However, the contribution of Fanconi-BRCA pathway deficiency to human T-cell acute lymphoblastic leukemia (T-ALL) remains undefined. We identified point mutations in 9 (23%) of 40 human T-ALL cases analyzed, with variant allele fractions consistent with heterozygous mutations early in tumor evolution. Two of these mutations were present in remission bone marrow specimens, suggesting germline alterations. BRCA2 was the most commonly mutated gene. The identified Fanconi-BRCA mutations encode hypomorphic or null alleles, as evidenced by their inability to fully rescue ...


Genome Editing Of Hbg1 And Hbg2 To Induce Fetal Hemoglobin, Jean-Yves Metais, Kevin Luk, Scot A. Wolfe, Shengdar Q. Tsai, Mitchell J. Weiss 2019 St. Jude Children's Research Hospital

Genome Editing Of Hbg1 And Hbg2 To Induce Fetal Hemoglobin, Jean-Yves Metais, Kevin Luk, Scot A. Wolfe, Shengdar Q. Tsai, Mitchell J. Weiss

Open Access Articles

Induction of fetal hemoglobin (HbF) via clustered regularly interspaced short palindromic repeats/Cas9-mediated disruption of DNA regulatory elements that repress gamma-globin gene (HBG1 and HBG2) expression is a promising therapeutic strategy for sickle cell disease (SCD) and beta-thalassemia, although the optimal technical approaches and limiting toxicities are not yet fully defined. We disrupted an HBG1/HBG2 gene promoter motif that is bound by the transcriptional repressor BCL11A. Electroporation of Cas9 single guide RNA ribonucleoprotein complex into normal and SCD donor CD34+ hematopoietic stem and progenitor cells resulted in high frequencies of on-target mutations and the induction of HbF to potentially ...


Rare Obscure Cause Of Upper Gastrointestinal Bleeding: Bleeding Duodenal Lymphangiectasia, Sufian Sorathia, Rulz Cantave, Vivek Choksi, Kairavee D. Dave, Steven Kaplan 2019 HCA Healthcare

Rare Obscure Cause Of Upper Gastrointestinal Bleeding: Bleeding Duodenal Lymphangiectasia, Sufian Sorathia, Rulz Cantave, Vivek Choksi, Kairavee D. Dave, Steven Kaplan

Gastroenterology

Introduction: While ulcerative and erosive mucosal changes are more commonly the culprit lesion of upper gastrointestinal bleeding, bleeding can also occur from traumatic, malignant or vascular lesions. An unusual and infrequent vascular cause of bleeding is from a focal intestinal lymphangiectasia (IL). We describe a patient who presented with bleeding from a duodenal IL.

Case Description/Methods: A 94-year-old female was sent by her nursing home for witnessed melena and new onset normocytic anemia with a hemoglobin of 7 g/dl. She was unable to provide a reliable history due to her advanced dementia. Her medical history was unknown. She ...


Iron Pill-Induced Gastropathy In Elderly Patients: A Case Series Report, Isin Y. Comba MD, Richard Henriquez, Sundeep Kumar, Ruthvik Srinvasa-Murthy, Maria Wallis-Crespo, Lakhinder Bhatia MD, Joshua Shultz 2019 HCA Healthcare

Iron Pill-Induced Gastropathy In Elderly Patients: A Case Series Report, Isin Y. Comba Md, Richard Henriquez, Sundeep Kumar, Ruthvik Srinvasa-Murthy, Maria Wallis-Crespo, Lakhinder Bhatia Md, Joshua Shultz

Gastroenterology

Introduction: Iron deficiency anemia is a global health problem with an estimated two billion cases worldwide. Oral iron supplementation is considered the first line treatment. However, recent studies favor the use of short-term IV iron especially in patients with drug tolerance and efficacy concerns. In this report, we present three cases of iron pill gastropathy in patient's whose clinical courses were complicated by gastric erosion, ulcer or gastrointestinal (GI) bleeding. All of our patients had a history of iron deficiency anemia and were being treated with oral ferrous sulfate.

Case Description/Methods: The first case involved a 72-year-old male ...


Guillain Barre Syndrome In A Patient With Sickle Cell Anemia, Kunjan Udani MD, Pooja Patel, Dveet Patel, Hajra Awwab, Nino Balanchivadze 2019 HCA Healthcare

Guillain Barre Syndrome In A Patient With Sickle Cell Anemia, Kunjan Udani Md, Pooja Patel, Dveet Patel, Hajra Awwab, Nino Balanchivadze

Internal Medicine

No abstract provided.


Sarcoidosis Presenting With Massive Splenomegaly And Severe Epistaxis, Austen Stoelting MD, Shawn B. Esperti DO, Andrew Mangano DO, Nino Balanchivadze MD 2019 HCA Healthcare

Sarcoidosis Presenting With Massive Splenomegaly And Severe Epistaxis, Austen Stoelting Md, Shawn B. Esperti Do, Andrew Mangano Do, Nino Balanchivadze Md

Internal Medicine

No abstract provided.


Treatment Patterns And Economic Burden Of Sickle-Cell Disease Patients Prescribed Hydroxyurea: A Retrospective Claims-Based Study, Nirmish Shah, Menaka Bhor, Lin Xie, Rashid Halloway, Steve Arcona, Jincy Paulose, Huseyin Yuce 2019 Duke University

Treatment Patterns And Economic Burden Of Sickle-Cell Disease Patients Prescribed Hydroxyurea: A Retrospective Claims-Based Study, Nirmish Shah, Menaka Bhor, Lin Xie, Rashid Halloway, Steve Arcona, Jincy Paulose, Huseyin Yuce

Publications and Research

Background: This study aimed to evaluate sickle-cell disease (SCD) treatment patterns and economic burden among patients prescribed hydroxyurea (HU) in the US, through claims data.

Methods: SCD patients with pharmacy claims for HU were selected from the Medicaid Analytic Extracts (MAX) from January 1, 2009 - December 31, 2013. The first HU prescription during the identification period was defined as the index date and patients were required to have had continuous medical and pharmacy benefits for ≥6 months baseline and 12 months follow-up periods. Patient demographics, clinical characteristics, treatment patterns, health care utilization, and costs were examined, and variables were analyzed ...


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