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Dmitry Karateev, Natalia Belokoneva
Physician awareness and perceptions towards biosimilars are important factors in their adoption to clinical practice. Our objectives were to assess levels of knowledge and attitudes towards biosimilars and key policies on their use among Russian physicians, define the level of interest in new information on biosimilars, and determine what evidence drives treatment decisions in Russia. Physicians with awareness of biologics across different specialties and regions of Russia completed an online survey. A Likert and other rating scales were used to collect opinions, which were summarized descriptively...
February 11, 2019: Biomolecules
Bruno C Medeiros, Bhavik J Pandya, Anna Hadfield, James Pike, Samuel Wilson, Cynthia Mueller, Cat N Bui, Scott C Flanders, Alex Rider, Laura E Horvath
OBJECTIVE: The aim of this analysis was to examine treatment patterns in patients with acute myeloid leukemia (AML) in routine clinical practice in the United States, including factors influencing the choice of front-line treatment intensity and the effect of age and treatment line. METHODS: We used data from the Adelphi AML Disease Specific Programme, a real-world, cross-sectional survey conducted in 2015. Physicians completed patient record forms providing patients' demographic and clinical characteristics...
February 2, 2019: Current Medical Research and Opinion
Luke Y C Chen, Andre Mattman, Michael A Seidman, Mollie N Carruthers
IgG4-related disease is a fibroinflammatory condition that can affect nearly any organ system. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. This review focuses on the hematological manifestations of IgG4-related disease, including lymphadenopathy, eosinophilia, and polyclonal hypergammaglobulinemia. The disease can easily be missed by unsuspecting hematologists, as patients may present with clinical problems that mimic disorders such as multicentric Castleman disease, lymphoma, plasma cell neoplasms and hypereosinophilic syndromes...
January 31, 2019: Haematologica
Roy Khalife, Anthony Grieco, Karima Khamisa, Alan Tinmouh, Chris McCudden, Elianna Saidenberg
BACKGROUND: Scurvy is a rare entity in developed countries and the diagnosis may often be delayed resulting in unnecessary investigations and/or potentially severe complications. A recent increase in the number of patients diagnosed with scurvy in our hematology clinics indicated the need to review the literature on the diagnosis and optimal management of similar patients. METHODS: We conducted a retrospective chart review of patients referred to hematology at our tertiary care centre between 2010 and 2018, who were ultimately diagnosed with scurvy...
January 24, 2019: Blood Cells, Molecules & Diseases
Georg Maschmeyer, Julien De Greef, Sibylle C Mellinghoff, Annamaria Nosari, Anne Thiebaut-Bertrand, Anne Bergeron, Tomas Franquet, Nicole M A Blijlevens, Johan A Maertens
A multitude of new agents for the treatment of hematologic malignancies has been introduced over the past decade. Hematologists, infectious disease specialists, stem cell transplant experts, pulmonologists and radiologists have met within the framework of the European Conference on Infections in Leukemia (ECIL) to provide a critical state-of-the-art on infectious complications associated with immunotherapeutic and molecular targeted agents used in clinical routine. For brentuximab vedotin, blinatumomab, CTLA4- and PD-1/PD-L1-inhibitors as well as for ibrutinib, idelalisib, HDAC inhibitors, mTOR inhibitors, ruxolitinib, and venetoclax, a detailed review of data available until August 2018 has been conducted, and specific recommendations for prophylaxis, diagnostic and differential diagnostic procedures as well as for clinical management have been developed...
January 30, 2019: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Shanmuganathan Chandrakasan, Sharat Chandra, Blachy J Davila Saldana, Troy R Torgerson, David Buchbinder
An array of monogenic immune defects marked by autoimmunity, lymphoproliferation, and hyperinflammation rather than infections have been described. Primary immune regulatory disorders pose a challenge to pediatric hematologists and oncologists. This paper focuses on primary immune regulatory disorders including autoimmune lymphoproliferative syndrome (ALPS) and ALPS-like syndromes, immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) and IPEX-like disorders, common variable immunodeficiency (CVID), CVID-like, and late-onset combined immunodeficiency (CID) disorders...
January 29, 2019: Pediatric Blood & Cancer
Ammar B Nassri, Valery Muenyi, Ahmad AlKhasawneh, Bruno De Souza Ribeiro, James S Scolapio, Miguel Malespin, Silvio W de Melo
BACKGROUND: A variety of immune-modulating drugs are becoming increasingly used for various cancers. Despite increasing indications and improved efficacy, they are often associated with a wide variety of immune mediated adverse events including colitis that may be refractory to conventional therapy. Although these drugs are being more commonly used by Hematologists and Oncologists, there are still many gastroenterologists who are not familiar with the incidence and natural history of gastrointestinal immune-mediated side effects, as well as the role of infliximab in the management of this condition...
January 21, 2019: World Journal of Gastrointestinal Pharmacology and Therapeutics
Ana G Almeida, António Almeida, Teresa Melo, Lurdes Guerra, Luís Lopes, Patrícia Ribeiro, Marta Duarte, Alexandra Mota, Ricardo Fontes-Carvalho
The use of tyrosine kinase inhibitors (TKIs) for the treatment of chronic myeloid leukemia has significantly altered the prognosis of this disease, enabling close to normal life expectancy. Despite their undeniable benefits, the use of TKIs is associated with an increased risk of side effects on the cardiovascular system, particularly of atherothrombotic events. It is therefore necessary to understand and prevent the adverse effects of these drugs, in order to enable antileukemic therapy to continue and to minimize patients' toxic exposure...
January 24, 2019: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
C Chabannon, R Bouabdallah, S Fürst, A Granata, C Saillard, N Vey, D Mokart, E Fougereau, C Lemarie, B Mfarrej, D Blaise, B Calmels
CAR-T cells are genetically modified human lymphocytes and gene therapy medicinal products. They are developed to treat cancers that express a membrane antigen targeted by the CAR. The FDA approved the two first-in-class medicinal products in 2017 and EMA in August 2018; both are autologous CAR-T cells targeting CD19 that is expressed at the surface of normal B-cells throughout their differentiation, and on B-cell lymphoid malignancies. Clinical efficacy was demonstrated for B-cell acute lymphoblastic leukemias, non-Hodgkin's lymphoma and chronic lymphocytic leukemia, although the marketing authorizations are less liberal in terms of indications...
January 24, 2019: La Revue de Médecine Interne
Zhuo Gao, Yini Wang, Jingshi Wang, Jia Zhang, Zhao Wang
The differentiation of primary hemophagocytic lymphohistiocytosis (pHLH) and macrophage activation syndrome (MAS) poses a challenge to hematologists. The aim of this study was (1) to compare the levels of soluble ST2 (sST2), sCD163, IFN-γ, IL-10, IL-18, TNF-α and Serum soluble interleukin-2 receptor (sCD25) in patients with pHLH and MAS and (2) to investigate whether they can help differentiate the two diseases. A total of 52 participants were recruited in this study, including 12 pHLH patients, 20 MAS patients, and 20 healthy subjects...
2019: Mediterranean Journal of Hematology and Infectious Diseases
Ellen K Ritchie, Dominick Latremouille-Viau, Annie Guerin, Irina Pivneva, Karen Habucky, Briana Ndife, George J Joseph, Ehab L Atallah
Tyrosine kinase inhibitor (TKI) therapy discontinuation practice in patients with chronic myeloid leukemia chronic phase (CML-CP) was assessed in real-world practice prior to the release of recommendations on discontinuation. Data were collected from US oncologists/hematologists (through web-based physician survey and patient chart review) on TKI therapy discontinuation practice including monitoring, adequate response for discontinuation, relapse, and symptoms following discontinuation. From the physician survey, 34% of oncologists/hematologists attempted discontinuation, with two-thirds doing so outside of a trial...
January 22, 2019: Leukemia & Lymphoma
Vincenzo De Sanctis, Ashraf T Soliman, Duran Canatan, Ploutarchos Tzoulis, Shahina Daar, Salvatore Di Maio, Heba Elsedfy, Mohamed A Yassin, Aldo Filosa, Nada Soliman, Karimi Mehran, Forough Saki, Praveen Sobti, Shruti Kakkar, Soteroula Christou, Alice Albu, Constantinos Christodoulides, Yurdanur Kilinc, Soad Al Jaouni, Doaa Khater, Saif A Alyaarubi, Su Han Lum, Saveria Campisi, Salvatore Anastasi, Maria Concetta Galati, Giuseppe Raiola, Yasser Wali, Ihab Z Elhakim, Demetris Mariannis, Vassilis Ladis, Christos Kattamis
In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged and been reported. As the early detection of these complications is essential for appropriate treatment and follow-up, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) promoted a survey on these complications in adult TM patients, among physicians (pediatricians, hematologists and endocrinologists) caring for TM patients in different countries...
January 15, 2019: Acta Bio-medica: Atenei Parmensis
Michelle Sholzberg
When faced with a life-threatening non-cancerous blood disorder, the term "benign" is a misnomer. Devastating diseases like catastrophic antiphospholipid antibody syndrome, acquired hemophilia, and severe immune thrombocytopenia present a challenge to the hematologist. They are often difficult to treat and lack appropriately powered, unbiased evidence to support management. Moreover, the label "benign" does a disservice as it subconsciously triggers discrepancies in prioritization for the care provider, the system, the patient and his/her family...
January 2019: Research and Practice in Thrombosis and Haemostasis
Marcello Tonelli, Natasha Wiebe, Braden J Manns, Scott W Klarenbach, Matthew T James, Pietro Ravani, Neesh Pannu, Jonathan Himmelfarb, Brenda R Hemmelgarn
Importance: Clinical experience suggests that there are substantial differences in patient complexity across medical specialties, but empirical data are lacking. Objective: To compare the complexity of patients seen by different types of physician in a universal health care system. Design, Setting, and Participants: Population-based retrospective cohort study of 2 597 127 residents of the Canadian province of Alberta aged 18 years and older with at least 1 physician visit between April 1, 2014 and March 31, 2015...
November 2, 2018: JAMA network open
Alexandru Martiniuc, Mugur Grasu, Radu Dumitru, Mihai Toma, Gina Rusu, Graţiela Manga, Ecaterina Scărlătescu, Bogdan Cotruţă, Melen Brâză, Răzvan Stoia, Vlad Herlea, Traian Dumitraşcu
Introduction: Gastric cancer is a rare cause of upper digestive hemorrhage. Associated co-morbidities may have a detrimental effect on both early and long-term outcomes after surgery for gastric cancer. Association of gastric adenocarcinoma with hemophilia A and angiodysplasia was not previously reported, and the impact on postoperative outcome is not known. Case Report: A 49-year-old male with inherited hemophilia A presented with upper digestive hemorrhage and severe anemia, and was diagnosed with gastric carcinoma...
November 2018: Chirurgia
Jorge E Cortes, Jane F Apperley, Daniel J DeAngelo, Michael W Deininger, Vamsi K Kota, Philippe Rousselot, Carlo Gambacorti-Passerini
Bosutinib, a BCR-ABL1 tyrosine kinase inhibitor (TKI), has been available for several years as a treatment for chronic-, accelerated-, and blast-phase chronic myeloid leukemia (CML), for patients with resistance or intolerance to prior therapy. In 2017, the BFORE trial demonstrated efficacy of bosutinib as first-line treatment in adult patients with newly diagnosed chronic-phase chronic myeloid leukemia (CP-CML). The most common adverse events (AEs) of any grade in bosutinib-treated patients in BFORE were diarrhea, nausea, thrombocytopenia, increased alanine aminotransferase, and increased aspartate aminotransferase, consistent with the most commonly reported AEs in earlier studies...
December 27, 2018: Journal of Hematology & Oncology
Amy E Caruso Brown, Katherine Frega
An eighteen-year-old with sickle cell disease was admitted to the pediatric hematology service at his local children's hospital for management of an acute pain crisis, one of many such admissions. He had a good relationship with his primary hematologist and primary nurse, but with other health care providers, there was evident friction. Sometimes, he was simply rude, rolling over and pretending to sleep in response to questions about his symptoms. When frustrated or convinced that his pain was not being addressed appropriately, he was prone to yelling and cursing at his nurses...
November 2018: Hastings Center Report
Li Li, Jill M Johnsen, Chau X Doan, Laurent A Bollag
Neuraxial anesthesia, as the standard of care for Cesarean deliveries, is associated with decreased blood loss. However, parturients with inherited bleeding disorders are at increased risk for epidural hematomas. A small retrospective study has shown that parturients with known factor deficiencies can safely undergo neuraxial anesthesia once the specific factors are replenished. We present a patient who had a considerably increased risk of peripartum bleeding from an unspecified inherited bleeding disorder and was provided a successful neuraxial anesthetic without complications...
2018: F1000Research
Zihai Li, Wenru Song, Mark Rubinstein, Delong Liu
The immune system is the hard-wired host defense mechanism against pathogens as well as cancer. Five years ago, we pondered the question if the era of cancer immunotherapy was upon us (Li et al., Exp Hem Oncol 2013). Exciting progresses have been made at all fronts since then, including (1) sweeping approval of six agents by the US Food and Drug Administration (FDA) to block the PD-1/PD-L1 pathway for treatment of 13 cancer types; (2) a paradigm shifting indication of PD-1 and CTLA4 blockers for the management of a broad class of cancers with DNA mismatch repair defect, the first-ever tissue agnostic approval of cancer drugs; (3) real world practice of adoptive T cell therapy with two CD19-directed chimeric antigen receptor T cell products (CAR-T) for relapsed and/or refractory B cell malignancies including acute lymphoid leukemia and diffuse large B cell lymphoma, signaling the birth of a field now known as synthetic immunology; (4) the award of 2018 Nobel Prize in Physiology and Medicine from the Nobel Committee to Tasuku Honjo and James Allison "for their discovery of cancer medicine by inhibition of negative immune regulation" ( www...
December 21, 2018: Journal of Hematology & Oncology
X Wei, M Zhao, Q Li, X Xiao, L Zhu
BACKGROUND: Calcineurin-inhibitor-induced pain syndrome (CIPS), a rare complication seen in patients with bone marrow transplants, is associated with the use of cyclosporine A (CsA) or tacrolimus (FK506). This case demonstrates the successful pain control of FK506-related CIPS in a 23-year-old male patient with previously reported characteristic clinical features of CIPS in addition to neuropathic symptoms and uncharacteristic imaging findings. On day 15 after the transplantation, the patient complained of severe pain in the lower limbs...
December 2018: Transplantation Proceedings
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