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400 full-text articles. Page 1 of 17.

Microrna199b Regulates Mouse Hematopoietic Stem Cells Maintenance, Aldona A. Karaczyn, Edward Jachimowicz, Jaspreet S. Kohli, Pradeep Sathyanarayana 2020 Maine Medical Center

Microrna199b Regulates Mouse Hematopoietic Stem Cells Maintenance, Aldona A. Karaczyn, Edward Jachimowicz, Jaspreet S. Kohli, Pradeep Sathyanarayana

Costas T. Lambrew Research Retreat 2020

The preservation of hematopoieticstem cell pool in bone marrow (BM) is crucial for sustained hematopoiesisin adults. Studies assessing adult hematopoieticstem cells functionality had been shown that for example loss of quiescence impairs hematopoieticstem cells maintenance. Although, miR‐199b is frequently down‐regulated in acute myeloid leukemia, its role in hematopoieticstem cells quiescence, self‐renewal and differentiation is poorly understood. Our laboratory investigated the role of miR‐199b in hematopoieticstem and progenitor cells (HSPCs) fate using miR‐199b‐5p global deletion mouse model. Characterization of miR‐199b expression pattern among normal HSPC populations revealed that miR‐199b is enriched in LT ...


Irisin Stimulates Osteoclast Differentiation And Function, Eben Estell, Phuong Le, Yosta Vegting, Hyeonwoo Kim, Roland Baron, Bruce Spiegelman, Clifford Rosen 2020 Maine Medical Center

Irisin Stimulates Osteoclast Differentiation And Function, Eben Estell, Phuong Le, Yosta Vegting, Hyeonwoo Kim, Roland Baron, Bruce Spiegelman, Clifford Rosen

Costas T. Lambrew Research Retreat 2020

Irisin is the cleaved product of the protein FNDC5 expressed in skeletal muscle, and is increased by exercise. Recent work suggests this myokine is a modulator of muscle-bone crosstalk. Irisin has been shown to increase bone formation (Colaiannia, PNAS 2015), prevent disuse-associated bone loss (Colaiannia, Sci Rep 2017), and directly stimulate osteoblasts (Colaiannia, Int J Endo 2014). These results demonstrate an anabolic function, but the full mechanisms of action on each key cell type in bone, and the relative impacts of irisin on bone formation, resorption, and coupled remodeling have yet to be elucidated. Converse to an anabolic role, genetic ...


Anticoagulation In The Very Old: Does Age Matter?, F. Jaspar Abu-Jaber, Kerri Barton, Caroline Knight, Frances Leslie Lucas, Robert Stein 2020 Pen Bay Medical Center

Anticoagulation In The Very Old: Does Age Matter?, F. Jaspar Abu-Jaber, Kerri Barton, Caroline Knight, Frances Leslie Lucas, Robert Stein

Costas T. Lambrew Research Retreat 2020

Background: There is a 4.5% annual risk of a thromboembolic stroke in patients with atrial fibrillation (AF) and a CHADS2-VASC score 2 or greater. Among these patients, anticoagulation using Warfarin or a direct oral anticoagulant has been shown to reduce the risk of events by 45%. However, there has been some provider concern about the increased risk of bleeding while on anticoagulation in patients who are older, frailer, and have multiple comorbidities. Previous studies have suggested that anticoagulation is recommended in nearly all patients with a CHADS2-VASC score at 2 or greater. Understanding factors that make patients poor candidates ...


Evaluation Of A Perioperative Anticoagulation Protocol In Renal Transplantation: A Retrospective Analysis., Sierra Ferreira, Brian Carlone, Marizela Savic 2020 Maine Medical Center

Evaluation Of A Perioperative Anticoagulation Protocol In Renal Transplantation: A Retrospective Analysis., Sierra Ferreira, Brian Carlone, Marizela Savic

Costas T. Lambrew Research Retreat 2020

Perioperative anticoagulation is standard for different surgical procedures in order to minimize the risk of thrombotic events. During renal transplantation, disturbances in hemostasis increase the likelihood for both thrombotic and hemorrhagic complications. This is detrimental to consider when balancing the prevention of thromboembolism including graft loss as well as major bleeding. In light of the literature lacking standardization, a perioperative anticoagulation protocol in renal transplantation was implemented at our institution in 2013. The primary purpose of this study is to determine the efficacy of the implemented protocol and further establish anticoagulation practices in the perioperative stage of renal transplantation. This ...


A Phase 1/2 Study Of The Oral Flt3 Inhibitor Pexidartinib In Relapsed/Refractory Flt3-Itd-Mutant Acute Myeloid Leukemia., Catherine C Smith, Mark J Levis, Olga Frankfurt, John M Pagel, Gail J Roboz, Richard M Stone, Eunice S Wang, Paul L Severson, Brian L West, Mai H Le, Sabine Kayser, Bao Lam, Henry H Hsu, Chao Zhang, Gideon Bollag, Alexander E Perl 2020 SWEDISH CANCER INSTITUTE.

A Phase 1/2 Study Of The Oral Flt3 Inhibitor Pexidartinib In Relapsed/Refractory Flt3-Itd-Mutant Acute Myeloid Leukemia., Catherine C Smith, Mark J Levis, Olga Frankfurt, John M Pagel, Gail J Roboz, Richard M Stone, Eunice S Wang, Paul L Severson, Brian L West, Mai H Le, Sabine Kayser, Bao Lam, Henry H Hsu, Chao Zhang, Gideon Bollag, Alexander E Perl

Articles, Abstracts, and Reports

FMS-like tyrosine kinase 3 (FLT3) tyrosine kinase inhibitors (TKIs) have activity in acute myeloid leukemia (AML) patients with FLT3 internal tandem duplication (ITD) mutations, but efficacy is limited by resistance-conferring kinase domain mutations. This phase 1/2 study evaluated the safety, tolerability, and efficacy of the oral FLT3 inhibitor PLX3397 (pexidartinib), which has activity against the FLT3 TKI-resistant F691L gatekeeper mutation in relapsed/refractory FLT3-ITD-mutant AML. Ninety patients were treated: 34 in dose escalation (part 1) and 56 in dose expansion (part 2). Doses of 800 to 5000 mg per day in divided doses were tested. No maximally tolerated dose ...


Iron Deficiency Anemia: An Unexpected Cause Of An Acute Occipital Lobe Stroke In An Otherwise Healthy Young Woman, Qian Zhang, Khine S. Shan, Conor O'Sullivan, Travis C. Nace 2020 Thomas Jefferson University

Iron Deficiency Anemia: An Unexpected Cause Of An Acute Occipital Lobe Stroke In An Otherwise Healthy Young Woman, Qian Zhang, Khine S. Shan, Conor O'Sullivan, Travis C. Nace

Abington Jefferson Health Papers

A 29-year-old caucasian woman who presented to the hospital with an acute onset of right eye visual disturbance and headache was found to have an acute left occipital lobe infarction. Past medical history was significant for iron deficiency anemia (IDA) secondary to menorrhagia. Her initial hemoglobin level was 7.8 G/DL, and her symptoms improved after iron and blood transfusions. Hypercoagulable studies were completed in the outpatient setting, and the results were unremarkable. Her acute stroke was most likely related to IDA as she had low cardiovascular risk factors along with a negative complete stroke workup.


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 ...


Extra Medullar Granulocytic Sarcoma: A Case Report Of An Exceptional Localization, Bouhani Malek, Sakhri Saida, Jaidane Olfa, Adouni Olfa, Chargui Riadh, Rahal Khaled 2020 SALAH AZAIZ INSTITUTE, DEPARTMENT OF ONCOLOGIC SURGERY, BOULEVARD 9TH APRIL, TUNIS 1009, TUNISIA

Extra Medullar Granulocytic Sarcoma: A Case Report Of An Exceptional Localization, Bouhani Malek, Sakhri Saida, Jaidane Olfa, Adouni Olfa, Chargui Riadh, Rahal Khaled

Journal of Mind and Medical Sciences

Granulocytic sarcoma is a rare type of tumor composed of extramedullary immature cells. The breast location is very rare; it accounts for less than 8% of cases. The present study reports the case of a 36-year-old female with a medical history of myelodysplastic syndrome. She was referred because of a lump in the left breast. We have diagnosed a case of granulocytic sarcoma of the breast by core biopsy. Histology and immunohistochemistry showed hypercellular smears with immature myeloid cells. The blast cells were myeloperoxidase positive.

The patient underwent a lumpectomy. Five months later, she developed a contralateral recurrence, treated by ...


Blood Test Shows High Accuracy In Detecting Stage I Non-Small Cell Lung Cancer., Cherylle Goebel, Christopher L Louden, Robert McKenna, Osita Onugha, Andrew Wachtel, Thomas Long 2020 Thoracic Surgery Department, John Wayne Cancer Institute, Santa Monica, California.

Blood Test Shows High Accuracy In Detecting Stage I Non-Small Cell Lung Cancer., Cherylle Goebel, Christopher L Louden, Robert Mckenna, Osita Onugha, Andrew Wachtel, Thomas Long

Articles, Abstracts, and Reports

BACKGROUND: In a previous study (Goebel et. al, Cancer Genomics Proteomics 16:229-244, 2019), we identified 33 biomarkers for an early stage (I-II) Non-Small Cell Lung Cancer (NSCLC) test with 90% accuracy, 80.3% sensitivity, and 95.4% specificity. For the current study, we used a narrowed ensemble of 21 biomarkers while retaining similar accuracy in detecting early stage lung cancer.

METHODS: A multiplex platform, 486 human plasma samples, and 21 biomarkers were used to develop and validate our algorithm which detects early stage NSCLC. The training set consisted of 258 human plasma with 79 Stage I-II NSCLC samples. The ...


Predictors Of Impending Acute Chest Syndrome In Patients With Sickle Cell Anaemia., Salam Alkindi, Ikhlas Al-Busaidi, Bushra Al-Salami, Samir Raniga, Anil Pathare, Samir K. Ballas 2020 Sultan Qaboos University Hospital

Predictors Of Impending Acute Chest Syndrome In Patients With Sickle Cell Anaemia., Salam Alkindi, Ikhlas Al-Busaidi, Bushra Al-Salami, Samir Raniga, Anil Pathare, Samir K. Ballas

Cardeza Foundation for Hematologic Research

Acute chest syndrome (ACS) is a major complication of sickle cell anaemia (SCA) and a leading cause for hospital admissions and death. We aimed to study the spectrum of clinical and laboratory features of ACS and to assess the predisposing factors and predictors of severity. A retrospective case-control cohort was studied by retrieving patient information from electronic medical records after ethical approval. One hundred adolescents and adults with SCA and hospital admissions for ACS were identified through the discharge summaries, along with 20 additional patients presenting with VOC, but without ACS (controls). Among the patients with ACS, fever (>38.5 ...


Grk6 Regulates The Hemostatic Response To Injury Through Its Rate-Limiting Effects On Gpcr Signaling In Platelets., Xi Chen, Shuchi Gupta, Matthew Cooper, Daniel DeHelian, Xuefei Zhao, Meghna U. Naik, Jeremy G.T. Wurtzel, Timothy J. Stalker, Lawrence E. Goldfinger, Jeffrey Benovic, Lawrence F. Brass, Steven E. McKenzie, Ulhas P. Naik, Peisong Ma 2020 Thomas Jefferson University

Grk6 Regulates The Hemostatic Response To Injury Through Its Rate-Limiting Effects On Gpcr Signaling In Platelets., Xi Chen, Shuchi Gupta, Matthew Cooper, Daniel Dehelian, Xuefei Zhao, Meghna U. Naik, Jeremy G.T. Wurtzel, Timothy J. Stalker, Lawrence E. Goldfinger, Jeffrey Benovic, Lawrence F. Brass, Steven E. Mckenzie, Ulhas P. Naik, Peisong Ma

Cardeza Foundation for Hematologic Research

G protein-coupled receptors (GPCRs) mediate the majority of platelet activation in response to agonists. However, questions remain regarding the mechanisms that provide negative feedback toward activated GPCRs to limit platelet activation and thrombus formation. Here we provide the first evidence that GPCR kinase 6 (GRK6) serves this role in platelets, using GRK6-/- mice generated by CRISPR-Cas9 genome editing to examine the consequences of GRK6 knockout on GPCR-dependent signaling. Hemostatic thrombi formed in GRK6-/- mice are larger than in wild-type (WT) controls during the early stages of thrombus formation, with a rapid increase in platelet accumulation at the site of injury ...


The Evolving Pharmacotherapeutic Landscape For The Treatment Of Sickle Cell Disease., Samir K Ballas 2020 Thomas Jefferson University

The Evolving Pharmacotherapeutic Landscape For The Treatment Of Sickle Cell Disease., Samir K Ballas

Cardeza Foundation for Hematologic Research

Sickle cell disease (SCD) is an extremely heterogeneous disease that has been associated with global morbidity and early mortality. More effective and inexpensive therapies are needed. During the last five years, the landscape of the pharmacotherapy of SCD has changed dramatically. Currently, 54 drugs have been used or under consideration to use for the treatment of SCD. These fall into 3 categories: the first category includes the four drugs (Hydroxyurea, L-Glutamine, Crizanlizumab tmca and Voxelotor) that have been approved by the United States Food and Drug Administration (FDA) based on successful clinical trials. The second category includes 22 drugs that ...


A Report From The Grndad Multi-Site Registry For Sickle Cell Disease: Iron Overload Is Under-Recognized And Under-Managed, Matthew Sears, PhD, Sophie Lanzkron, MD, MHS, Carolyn Hoope, MD, Joshua J. Field, MD, E. Leila Jerome Clay, MD, MCTS, FAAP, Susan Padrino, MD, Payal C. Desai, MD, Lynne D. Neumayr, Deepa Manwani, MD, Jane Little, MD 2020 Thomas Jefferson University

A Report From The Grndad Multi-Site Registry For Sickle Cell Disease: Iron Overload Is Under-Recognized And Under-Managed, Matthew Sears, Phd, Sophie Lanzkron, Md, Mhs, Carolyn Hoope, Md, Joshua J. Field, Md, E. Leila Jerome Clay, Md, Mcts, Faap, Susan Padrino, Md, Payal C. Desai, Md, Lynne D. Neumayr, Deepa Manwani, Md, Jane Little, Md

Phase 1

Introduction: GRNDaD is a prospective registry for people with SCD that opened to enrollment in 2016. Nine comprehensive SCD centers from across the United States are currently enrolling patients. The registry includes iron status and management data, important in SCD because chronic transfusion therapy is a mainstay of prophylactic management. Each unit of transfused blood introduces approximately 250 mg of iron into the blood, which can lead to systemic iron deposition, and untreated may lead to organ dysfunction or death.

Methods: GRNDaD currently contains prospective baseline and annual update information on approximately 1000 people with SCD. We analyzed ferritin levels ...


The Efficacy Of Acute Normovolemic Hemodilution Compared To Cell Salvage As A Blood Transfusion Alternative, Julia Yu 2020 Arcadia University

The Efficacy Of Acute Normovolemic Hemodilution Compared To Cell Salvage As A Blood Transfusion Alternative, Julia Yu

Capstone Showcase

Blood transfusions are currently the treatment of choice for significant blood loss. However, there are a variety of reasons for which they cannot be utilized. Current alternatives are acute normovolemic hemodilution (ANH) and cell 1 This study analyzes whether ANH or cell salvage is more effective in minimizing blood loss in perioperative or trauma patients. Multiple different studies involving either ANH or cell salvage were compared to each other. However, due to the lack of research conducted in either method, results obtained were largely inconclusive.


Quiet A Puzzle: A Case Report Of 15 Year Old Female With Severe Anemia Due To Scurvy, Shaikha Alqahtani, Wang Zhihong 2020 Children hospital of Richmond

Quiet A Puzzle: A Case Report Of 15 Year Old Female With Severe Anemia Due To Scurvy, Shaikha Alqahtani, Wang Zhihong

Graduate Medical Education (GME) Resident and Fellow Research Day Posters

Scurvy is historically thought of a disease affecting sailors in the seventeenth century. Vitamin C deficiency is rare in the developed world. It is mainly found in patients with poor nutrition and specifically those with developmental delay. The spectrum of scurvy is varied and includes dermatological, dental, bone and systemic manifestations. We present to you a case of a 15 year old female with a history of trisomy 21 presenting with severe anemia as a direct cause of vitamin C deficiency.

She presented with a one month history of progressive bilateral lower extremity bruising and inability to walk. Physical exam ...


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 ...


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.


Plasma Cell Leukemia Mimicking Hairy Cell Leukemia: Extended Role Of Immunophenotyping In Correct Diagnosis, Ayesha Majeed Memon, Natasha Ali 2019 Aga Khan University

Plasma Cell Leukemia Mimicking Hairy Cell Leukemia: Extended Role Of Immunophenotyping In Correct Diagnosis, Ayesha Majeed Memon, Natasha Ali

Department of Pathology and Laboratory Medicine

No abstract provided.


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 ...


Secretion Of A Heparin-Like Anticoagulant (Hlac) In Plasma Cell Neoplasia, Kevin J. Hess DO, Joseph Moran DO, Rachel Kinney DO 2019 Lehigh Valley Health Network

Secretion Of A Heparin-Like Anticoagulant (Hlac) In Plasma Cell Neoplasia, Kevin J. Hess Do, Joseph Moran Do, Rachel Kinney Do

Department of Medicine

No abstract provided.


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