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Hematology

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284 papers 500 to 1000 followers
By Ronald Lands Benign hematologist
https://www.readbyqxmd.com/read/30486249/iron-as-a-therapeutic-target-in-hfe-related-hemochromatosis-usual-and-novel-aspects
#1
REVIEW
Olivier Loréal, Thibault Cavey, François Robin, Moussa Kenawi, Pascal Guggenbuhl, Pierre Brissot
Genetic hemochromatosis is an iron overload disease that is mainly related to the C282Y mutation in the HFE gene. This gene controls the expression of hepcidin, a peptide secreted in plasma by the liver and regulates systemic iron distribution. Homozygous C282Y mutation induces hepcidin deficiency, leading to increased circulating transferrin saturation, and ultimately, iron accumulation in organs such as the liver, pancreas, heart, and bone. Iron in excess may induce or favor the development of complications such as cirrhosis, liver cancer, diabetes, heart failure, hypogonadism, but also complaints such as asthenia and disabling arthritis...
November 26, 2018: Pharmaceuticals
https://www.readbyqxmd.com/read/30462941/oligonucleotide-drugs-for-transthyretin-amyloidosis
#2
LETTER
Joel N Buxbaum
New England Journal of Medicine, Volume 379, Issue 21, Page 2085-2086, November 2018.
November 22, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/30476292/cardiac-amyloidosis
#3
Chenghong Yu, Yuehua Lu, Jianhong Lu
No abstract text is available yet for this article.
November 22, 2018: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/12604412/prevalence-of-hereditary-hyperferritinemia-cataract-syndrome-in-blood-donors-and-patients-with-cataract
#4
LETTER
Claudia Bozzini, Silvia Galbiati, Elisa Tinazzi, Raffaella Aldigeri, Giovanna De Matteis, Domenico Girelli
No abstract text is available yet for this article.
February 2003: Haematologica
https://www.readbyqxmd.com/read/30419140/oncology-in-transition-changes-challenges-and-opportunities
#5
Deborah Schrag, Ethan Basch
No abstract text is available yet for this article.
November 8, 2018: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/30337321/walking-on-myeloma
#6
Bart Barlogie, Joshua Richter
No abstract text is available yet for this article.
October 18, 2018: Blood
https://www.readbyqxmd.com/read/30267448/venous-thrombosis-in-unusual-sites-a-practical-review-for-the-hematologist
#7
REVIEW
Joseph J Shatzel, Matthew O'Donnell, Sven R Olson, Matthew R Kearney, Molly M Daughety, Justine Hum, Khanh P Nguyen, Thomas G DeLoughery
Thrombosis of unusual venous sites encompasses a large part of consultative hematology and is encountered routinely by practicing hematologists. Contrary to the more commonly encountered lower extremity venous thrombosis and common cardiovascular disorders, the various thromboses outlined in this review have unique presentations, pathophysiology, workup, and treatments that all hematologists should be aware of. This review attempts to outline the most up to date literature on cerebral, retinal, upper extremity, hepatic, portal, splenic, mesenteric, and renal vein thrombosis, focusing on the incidence, pathophysiology, provoking factors, and current recommended treatments for each type of unusual thrombosis to provide a useful and practical review for the hematologist...
September 28, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/30206117/how-i-manage-medical-complications-of-%C3%AE-thalassemia-in-adults
#8
REVIEW
Ali T Taher, Maria Domenica Cappellini
The complex pathophysiology in β-thalassemia can translate to multiple morbidities that affect every organ system. Improved survival due to advances in management means that patients are exposed to the harmful effects of ineffective erythropoiesis, anemia, and iron overload for a longer duration, and we started seeing new or more frequent complications in adult compared with younger patients. In this article, we highlight particular aspects of managing adult patients with β-thalassemia, using our own experience in treating such patients...
October 25, 2018: Blood
https://www.readbyqxmd.com/read/30181422/chronic-myeloid-leukemia-version-1-2019-nccn-clinical-practice-guidelines-in-oncology
#9
Jerald P Radich, Michael Deininger, Camille N Abboud, Jessica K Altman, Ellin Berman, Ravi Bhatia, Bhavana Bhatnagar, Peter Curtin, Daniel J DeAngelo, Jason Gotlib, Gabriela Hobbs, Madan Jagasia, Hagop M Kantarjian, Lori Maness, Leland Metheny, Joseph O Moore, Arnel Pallera, Philip Pancari, Mrinal Patnaik, Enkhtsetseg Purev, Michal G Rose, Neil P Shah, B Douglas Smith, David S Snyder, Kendra L Sweet, Moshe Talpaz, James Thompson, David T Yang, Kristina M Gregory, Hema Sundar
Chronic myeloid leukemia (CML) is defined by the presence of Philadelphia chromosome (Ph), resulting from a reciprocal translocation between chromosomes 9 and 22 [t(9;22] that gives rise to a BCR-ABL1 fusion gene. CML occurs in 3 different phases (chronic, accelerated, and blast phase) and is usually diagnosed in the chronic phase. Tyrosine kinase inhibitor (TKI) therapy is a highly effective first-line treatment option for all patients with newly diagnosed chronic phase CML (CP-CML). The selection TKI therapy should be based on the risk score, toxicity profile of TKI, patient's age, ability to tolerate therapy, and the presence of comorbid conditions...
September 2018: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/30153096/hydroxycarbamide-plus-aspirin-versus-aspirin-alone-in-patients-with-essential-thrombocythemia-age-40-to-59-years-without-high-risk-features
#10
Anna L Godfrey, Peter J Campbell, Cathy MacLean, Georgina Buck, Julia Cook, Julie Temple, Bridget S Wilkins, Keith Wheatley, Jyoti Nangalia, Jacob Grinfeld, Mary Frances McMullin, Cecily Forsyth, Jean-Jacques Kiladjian, Anthony R Green, Claire N Harrison
Purpose Cytoreductive therapy is beneficial in patients with essential thrombocythemia (ET) at high risk of thrombosis. However, its value in those lacking high-risk features remains unknown. This open-label, randomized trial compared hydroxycarbamide plus aspirin with aspirin alone in patients with ET age 40 to 59 years and without high-risk factors or extreme thrombocytosis. Patients and Methods Patients were age 40 to 59 years and lacked a history of ischemia, thrombosis, embolism, hemorrhage, extreme thrombocytosis (platelet count ≥ 1,500 × 109 /L), hypertension, or diabetes requiring therapy...
August 28, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/30099106/when-to-resume-direct-oral-anticoagulants-following-gastrointestinal-bleeding
#11
EDITORIAL
Moe H Kyaw, Francis K L Chan
No abstract text is available yet for this article.
August 9, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/30084010/predictors-of-clinical-responses-to-hypomethylating-agents-in-acute-myeloid-leukemia-or-myelodysplastic-syndromes
#12
REVIEW
Hong Wang, Yan Li, Na Lv, Yonghui Li, Lili Wang, Li Yu
Azacitidine and decitabine, two hypomethylating agents, are known to be effective in the treatment of high-risk myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML) patients who cannot endure intensive cytotoxic chemotherapy or are not eligible for transplantation. However, the treatment response rate is low. The molecular mechanisms underlying the resistance to demethylation therapy are unclear. Though a wide range of predictors of treatment response have been investigated, no consensus has been reached...
November 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29112667/pica-in-pregnancy-an-unusual-presentation
#13
Katharine E Epler, Arand Pierce, Valerie J Rappaport
BACKGROUND: Pica is common in pregnancy and is often felt to be benign. The following case of severe pica presenting without anemia is unusual in its presentation, laboratory findings, and treatment. CASE: A 31-year-old multiparous woman at 37 0/7 weeks of gestation presented with esophagitis and gastritis secondary to laundry detergent consumption. She had borderline anemia (hemoglobin of 11 g/dL and hematocrit of 37%, mean corpuscular volume 80%) but was severely iron-deficient (serum ferritin 7 micrograms/dL)...
December 2017: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27184678/iron-deficiency-anaemia-in-pregnancy-the-role-of-parenteral-iron
#14
REVIEW
Umo I Esen
Maternal and perinatal morbidity and mortality remain major challenges in the delivery of safe maternity care worldwide. Anaemia in pregnancy is an important contributor to this dismal picture, especially where blood transfusion services are poorly developed. An early diagnosis and treatment of iron deficiency anaemia in pregnancy using the new generation dextran-free parenteral iron preparations can save lives and reduce morbidity in selected pregnancies. It is time to cast aside the fears associated with the use of the old parenteral iron preparations which were associated a high incidence of anaphylaxis, and embrace the use of new parenteral iron products which have better side effect profiles and can deliver total dose infusions without the need for test dosing...
January 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/30042411/dysregulated-iron-metabolism-in-polycythemia-vera-etiology-and-consequences
#15
REVIEW
Yelena Z Ginzburg, Maria Feola, Eran Zimran, Judit Varkonyi, Tomas Ganz, Ronald Hoffman
Polycythemia vera (PV) is a chronic myeloproliferative neoplasm. Virtually all PV patients are iron deficient at presentation and/or during the course of their disease. The co-existence of iron deficiency and polycythemia presents a physiological disconnect. Hepcidin, the master regulator of iron metabolism, is regulated by circulating iron levels, erythroblast secretion of erythroferrone, and inflammation. Both decreased circulating iron and increased erythroferrone levels, which occur as a consequence of erythroid hyperplasia in PV, are anticipated to suppress hepcidin and enable recovery from iron deficiency...
October 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29946191/diagnostic-algorithm-for-lower-risk-myelodysplastic-syndromes
#16
REVIEW
Ghulam J Mufti, Donal P McLornan, Arjan A van de Loosdrecht, Ulrich Germing, Robert P Hasserjian
Rapid advances over the past decade have uncovered the heterogeneous genomic and immunologic landscape of myelodysplastic syndromes (MDS). This has led to notable improvements in the accuracy and timing of diagnosis and prognostication of MDS, as well as the identification of possible novel targets for therapeutic intervention. For the practicing clinician, however, this increase in genomic, epigenomic, and immunologic knowledge needs consideration in a "real-world" context to aid diagnostic specificity...
August 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/30055055/aplastic-anemia-etiology-molecular-pathogenesis-and-emerging-concepts
#17
REVIEW
Rory M Shallis, Rami Ahmad, Amer M Zeidan
Aplastic anemia (AA) is rare disorder of bone marrow failure which if severe and not appropriately treated is highly fatal. AA is characterized by morphologic marrow features, namely hypocellularity, and resultant peripheral cytopenias. The molecular pathogenesis of AA is not fully understood, and a uniform process may not be the culprit across all cases. An antigen-driven and likely autoimmune dysregulated T-cell homeostasis is implicated in the hematopoietic stem cell injury which ultimately founds the pathologic features of the disease...
December 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/30012636/monoclonal-gammopathy-of-clinical-significance-a-novel-concept-with-therapeutic-implications
#18
REVIEW
Jean-Paul Fermand, Frank Bridoux, Angela Dispenzieri, Arnaud Jaccard, Robert A Kyle, Nelson Leung, Giampaolo Merlini
Monoclonal gammopathy is a common condition, particularly in the elderly. It can indicate symptomatic multiple myeloma or another overt malignant lymphoid disorder requiring immediate chemotherapy. More frequently, it results from a small and/or quiescent secreting B-cell clone, is completely asymptomatic, and requires regular monitoring only, defining a monoclonal gammopathy of unknown significance (MGUS). Sometimes, although quiescent and not requiring any treatment per se, the clone is associated with potentially severe organ damage due to the toxicity of the monoclonal immunoglobulin or to other mechanisms...
October 4, 2018: Blood
https://www.readbyqxmd.com/read/16785130/corticosteroids-can-reverse-severe-imatinib-induced-hepatotoxicity
#19
Dario Ferrero, Enrico Maria Pogliani, Giovanna Rege-Cambrin, Carmen Fava, Giovanna Mattioli, Chiara Dellacasa, Elisabetta Campa, Paola Perfetti, Monica Fumagalli, Mario Boccadoro
BACKGROUND: Imatinib can induce severe hepatotoxicity, in 1-5% of CML patients, many of whom need permanent imatinib discontinuation. DESIGN AND RESULTS: We report 5 CML patients who developed grade 3-4 hepatotoxicity after 2-8 months in imatinib. Different aetiologies of liver damage were ruled out and toxicity recurred in 2 patients with further attempts at low dose imatinib. In all patients prednisone or methylprednisolone at 25- 40 mg/day resolved hepatotoxicity in 3-8 weeks and allowed imatinib to be resumed at full doses...
June 2006: Haematologica
https://www.readbyqxmd.com/read/28040690/obesity-and-the-transformation-of-monoclonal-gammopathy-of-undetermined-significance-to-multiple-myeloma-a-population-based-cohort-study
#20
Su-Hsin Chang, Suhong Luo, Theodore S Thomas, Katiuscia K O'Brian, Graham A Colditz, Nils P Carlsson, Kenneth R Carson
Background: Multiple myeloma (MM) is one of the most common hematologic malignancies in the United States and is consistently preceded by monoclonal gammopathy of undetermined significance (MGUS). This study investigates the role of obesity in the progression of MGUS to MM. Methods: A retrospective identified cohort of patients in the US Veterans Health Administration database diagnosed with MGUS between October 1, 1999, and December 31, 2009, was followed through August 6, 2013...
May 2017: Journal of the National Cancer Institute
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