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Rory M Shallis, Mina L Xu, Nikolai A Podoltsev, Susanna A Curtis, Bryden T Considine, Suchin R Khanna, Alexa J Siddon, Amer M Zeidan
In patients suspected to have myelodysplastic syndrome (MDS), especially in those patients without cytogenetic abnormalities or blast excess, accurate morphologic review by an expert hematopathologist and meticulous exclusion of other secondary causes of myelodysplasia are vital to establish the diagnosis. Errors in diagnosis can lead to dangerous consequences such as the administration of hypomethylating agents, lenalidomide, or even the use of intensive chemotherapy or allogeneic hematopoietic cell transplantation in patients who do not have an underlying MDS or even a malignant hematopoietic process...
August 14, 2018: Annals of Hematology
Sisi Li, Diying Shen, Xiaoping Guo, Chan Liao, Yongmin Tang
Antibody-targeting therapy has drawn great interests to the hematologists and oncologists. 3A4, a novel antibody recognizing human CD45RA antigen, is a new target molecule for leukemias and holds a therapeutic potential for myeloid lineage leukemias. However, murine antibodies cannot be safely used in patients because of their strong immune reaction, humanization of the antibodies interested will be an important development step for therapeutic purpose. The aim of this study was to engineer the mouse 3A4 and to investigate the biological activity of its chimeric form...
August 10, 2018: DNA and Cell Biology
Bradley W Blaser, Leonard I Zon
Generating an HSC in vitro from non-hematopoietic tissue has been a goal of experimental hematologists for decades. Until recently, no in vitro-derived cell has closely demonstrated the full lineage potential and self-renewal capacity of a true HSC. Studies revealing stem cell ontogeny from embryonic mesoderm to hemogenic endothelium to HSC provided the key to inducing HSC-like cells in vitro from a variety of cell types. Here we review the path to this discovery and discuss the future of autologous transplantation with in vitro-derived HSCs as a therapeutic modality...
August 8, 2018: Blood
Laura Rodriguez-Romo, Alberto Olaya Vargas, Sumit Gupta, Jaime Shalkow-Klincovstein, Lourdes Vega-Vega, Alfonso Reyes-Lopez, Carlo Cicero-Oneto, Juan Mejia-Arangure, Oscar Gonzalez-Ramella, Rafael Pineiro-Retif, Aracely Lopez-Facundo, Maria de Los Angeles Del Campo-Martinez, Isidoro Tejocote, Kelly Brennan, Christopher M Booth
Purpose Limited data describe the delivery of pediatric cancer care in Mexico. We report a nationwide survey of pediatric cancer units. Methods An electronic survey was distributed to 74 pediatric cancer units in Mexico to describe case volumes; organization of care; and availability of medical/surgical specialists, supportive care, complex therapies, and diagnostic services. Centers were classified as low (< 30 new patients/year), medium (30 to 59/year) and high (≥ 60/year). Results Sixty-two centers completed the survey (response rate, 84%)...
July 2018: Journal of Global Oncology
Sarah A Hall, Emily Riehm Meier, Sweta Gupta, Charles Nakar, Angeli Rampersad, Nihal Bakeer, Brandon Hardesty, Manjusha Kumar
We present five patients with coexistent von Willebrand disease (VWD) and Ehlers-Danlos syndrome and 21 with VWD and joint hypermobility. Females outnumbered males ten to three, Beighton scores were documented in 58% (15 of 26 patients), and several patients experienced moderately severe bleeding. We believe coexistent hypermobility disorder with VWD potentially affects bleeding severity and want to raise awareness among hematologists. Evaluation by geneticists is recommended because of the varying complexities observed across the disease spectrum, and the availability of new genetic tests should lead to more accurate diagnoses for the various hypermobility disorders...
August 7, 2018: Pediatric Blood & Cancer
Mariam L Krikorian, Amanda S Growdon, Alyna T Chien
BACKGROUND: A key juncture in patient hospitalization is determining which type of physician should be primarily responsible for directing treatment. We (1) examine the frequency hospitalists and subspecialists agree on preferred assignments and (2) compare preferred assignment with actual assignment. METHODS: Using a mixed methods approach, we first surveyed 66 physicians in 8 specialties about hospitalist assignments versus subspecialist assignments for 176 diagnoses at an academic children's hospital...
August 2018: Hospital Pediatrics
Stacy E Croteau, Michael U Callaghan, Joanna Davis, Amy L Dunn, Michael Guerrera, Osman Khan, Ellis J Neufeld, Leslie J Raffini, Michael Recht, Michael Wang, Alfonso Iorio
Background: Emergence of population pharmacokinetic models for prediction of individual pharmacokinetic (PK) profiles facilitates individualization of prescribed prophylactic therapy for patients with hemophilia A and B and may have a favorable impact on clinical outcomes and annual factor utilization. How providers approach the integration and application of these data into routine clinical practice is not clear. Objective: To explore the potential application of and barriers to incorporating PK profiles into current hemophilia prophylaxis decision making...
July 2018: Research and Practice in Thrombosis and Haemostasis
Joyce C M Lam, M Joseph John, Alison Street
Background: Poor disease understanding and gaps in expertise regarding hemophilia care have been identified at all levels in Asia Pacific. Continued education for involved healthcare professionals (HCPs) is crucial for improved delivery. Objectives: To identify training and educational needs of hemophilia HCPs in Asia Pacific. Methods: Clinicians working at hemophilia treatment centers (HTCs), identified from the World Federation of Hemophilia Directory, were contacted by the Asia Pacific Hemophilia Working Group (APHWG)...
July 2018: Research and Practice in Thrombosis and Haemostasis
V M White, G Skaczkowski, R Pinkerton, M Coory, M Osborn, H Bibby, W Nicholls, L M Orme, R Conyers, M B Phillips, R Harrup, R Walker, K Thompson, A Anazodo
BACKGROUND: While several studies have examined the treatment of adolescents and young adults (AYAs) with acute lymphoblastic leukemia (ALL), studies of acute myeloid leukemia (AML) are rare. Using national data for Australia, we describe (i) the number and type of treatment centers caring for AYAs, (ii) induction/first-line treatments, and (iii) survival outcomes. PROCEDURE: National population-based study assessing treatment of 15- to 24-year-olds diagnosed with ALL or AML between 2007 and 2012...
July 24, 2018: Pediatric Blood & Cancer
Geremiha Emerson, Colin G Kaide
Emergency providers are likely to encounter patients with acute and chronic leukemias. In some cases, the first presentation to the emergency department may be for symptoms related to blast crisis and leukostasis. Making a timely diagnosis and consulting a hematologist can be life saving. Presenting symptoms are caused by complications of bone marrow infiltration and hyperleukocytosis with white blood cell counts over 100,000. Presentations may include fatigue (anemia), bleeding (thrombocytopenia), shortness of breath, and/or neurologic symptoms owing to hyperleukocytosis and subsequent leukostasis...
August 2018: Emergency Medicine Clinics of North America
Kwok-Man Tong, Jiaan-Der Wang, Shin-Tsu Chang, Yuan-Yang Cheng, Shian-Shiang Wang
Perioperative management of persons with hemophilia (PWH) is a challenge for surgeons and hematologists. Reductions in mortality rate and complications have been achieved since the introduction of clotting factor concentrates (CFCs), which improve hemostatic control. However, there is no clear consensus on the optimal dosing of CFC administration. The aim of this study was to evaluate the outcome of PWH without inhibitors in patients undergoing invasive or surgical procedures. A total of 161 procedures, including 57 major and 104 minor ones were retrospectively reviewed...
July 12, 2018: Journal of the Chinese Medical Association: JCMA
Pier Carmine Passarelli, Paolo De Angelis, Guido Pasquantonio, Paolo Francesco Manicone, Fernando Verdugo, Antonio D'Addona
PURPOSE: The aim of the present study was to analyze the management of single dental extractions and postoperative bleeding in patients with a diagnosis of factor V deficiency. A careful evaluation of each case will allow the team to categorize the risk and operate safely, minimizing the incidence of intraoperative and postoperative complications. If necessary, the oral-maxillofacial surgeon can choose to do so in collaboration with the hematologist on a case-by-case basis. PATIENTS AND METHODS: The present retrospective study included 5 patients with mild congenital factor V deficiency who had undergone at least 1 dental extraction...
June 21, 2018: Journal of Oral and Maxillofacial Surgery
Pierre Bories, Sébastien Lamy, Célestine Simand, Sarah Bertoli, Cyrille Delpierre, Sandra Malak, Luc Fornecker, Stéphane Moreau, Christian Récher, Antoine Nebout
Elderly patients with acute myeloid leukemia can be treated with intensive chemotherapy, low-intensity therapy such as low-dose aracytine or hypomethylating agents, or best supportive care. The choice between these treatments is a function of many patient-related and physician-related factors. We investigated how physicians' behavioral characteristics, in particular their attitudes towards risk and uncertainty affect medical decision-making between intensive and non-intensive therapy in this setting. A nationwide cross-sectional online survey of hematologists collected data on medical decision-making for six clinical vignettes involving older acute myeloid leukemia patients that were representative of routine practice...
July 13, 2018: Haematologica
Marianna Politou, Giorgos Dryllis, Maria Efstathopoulou, Serena Valsami, Faidra-Evangelia Triantafyllou, Athanasia Tsaroucha, Antonios Kattamis, Nikos F Vlahos
Thalassemia intermedia (TI) is a clinical definition which represents a wide spectrum of thalassemia genotypes but mainly includes patients who do not require or only occasionally require transfusion. An uncommon case of a 32-year-old Greek woman, para 1, at the 22nd week + day 3 of gestation with thalassemia intermedia (she was splenectomized), where her pregnancy was complicated with portal vein thrombosis, splenic thrombosis, and partial HELLP, is described. This is a generally uncommon event in thalassemia intermedia...
2018: Case Reports in Obstetrics and Gynecology
Alice Vianello, Elisa Vencato, Maurizio Cantini, Giovanni Zanconato, Erminia Manfrin, Alberto Zamo, Francesco Zorzi, Filippo Mazzi, Nicola Martinelli, Elena Cavaliere, Francesca Monari, Donatella Venturelli, Francesca Ferrara, Oliviero Olivieri, Lucia De Franceschi
BACKGROUND: The desire for pregnancy in sickle cell disease (SCD) women has become a true challenge for hematologists, requiring a multidisciplinary approach. Erythrocytapheresis (ECP) is an important therapeutic tool in SCD, but only limited data on starting time and the effects of ECP during pregnancy are available. STUDY DESIGN AND METHODS: This is a double-center retrospective cross-sectional study on a total of 46 single pregnancies in SCD women from January 2008 to June 2017...
July 8, 2018: Transfusion
Gregory M T Guilcher, Tony H Truong, Santosh L Saraf, Jacinth J Joseph, Damiano Rondelli, Matthew M Hsieh
Sickle cell disease (SCD) chronically damages multiple organs over the lifetime of affected individuals. Allogeneic hematopoietic cell transplantation (allo-HCT) is the most studied curative intervention. Fully matched related marrow, peripheral blood derived, or cord blood HCT have the best transplant outcome for symptomatic patients with SCD. For patients with asymptomatic or milder disease who have this donor option available, risks and benefits of HCT should be discussed among the patient, family, treating hematologist, and transplant physician, and decision to proceed to HCT should be individualized...
April 2018: Seminars in Hematology
Kate M Fennell, Luke Bamford, Ian Olver, Carlene J Wilson
Rates of referral of patients to psychosocial services are low in most cancer treatment centers, while rates of distress are high. The purpose of this study is to identify clinicians' barriers to referring cancer patients to psychosocial services and strategies that could increase rates of referral. A purposive sampling method ensured data were gathered in two large public teaching hospitals from seven oncologists and five hematologists with varying levels of experience, of whom five were female. Data were collected using semistructured interviews guided by the Capability, Opportunity, Motivation and Behavior model (Michie S, Atkins L, West R...
June 28, 2018: Translational Behavioral Medicine
Guido Finazzi, Valerio De Stefano, Tiziano Barbui
Myeloproliferative neoplasms (MPNs) are a leading cause of splanchnic vein thrombosis (SVT). SVT is observed in all MPNs and frequently affects young patients. Therapy should be addressed to three main goals: preventing thrombosis recurrence, managing the underlying MPN, and supporting liver dysfunction. Life-long oral anticoagulation with vitamin K antagonists is the cornerstone of the antithrombotic treatment. However, recurrences of SVT or other thrombosis may occur in 15-20% of patients. Direct oral anticoagulants can represent an alternative and preliminary data encourage comparative studies...
June 26, 2018: Blood Cancer Journal
M Kelsey Kirkwood, Amy Hanley, Suanna S Bruinooge, Elizabeth Garrett-Mayer, Laura A Levit, Caroline Schenkel, Jerome E Seid, Blase N Polite, Richard L Schilsky
PURPOSE: To describe the US hematology and medical oncology practice landscape and to report findings of the sixth annual ASCO Oncology Practice Census survey. PARTICIPANTS AND METHODS: ASCO used Medicare Physician Compare data to characterize oncology practices in the United States. Practice size, number of care sites, and geographic distribution were determined. Trends in the number and size of practices from 2013 to 2017 were examined. All US oncology practices were targeted for the survey; survey responses were linked to the practices identified from Physician Compare to augment results and assess generalizability...
July 2018: Journal of Oncology Practice
Shin Yeu Ong, Shao-Tzu Li, Gee Chuan Wong, Aloysius Yew Leng Ho, Chandramouli Nagarajan, Joanne Ngeow
Inherited bone marrow failure syndrome (IBMFS) including Shwachman Diamond Syndrome (SDS) can present initially to the hematologist with myelodysplastic syndrome (MDS). Accurate diagnosis affects choice of chemotherapy, donor selection, and transplant conditioning. We report a case of delayed diagnosis of SDS in a family with another child with aplastic anemia, and review reported cases of SDS in Asia. This highlights the gap in identifying inherited bone marrow failure syndromes in adults with hematologic malignancies...
2018: Leukemia Research Reports
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