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Hematology

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216 papers 100 to 500 followers
By Ronald Lands Benign hematologist
https://www.readbyqxmd.com/read/28295192/state-of-the-art-how-i-manage-immune-thrombocytopenia
#1
REVIEW
Nichola Cooper
The management of patients with immune thrombocytopenia (ITP) is rapidly evolving. Over the last 15 years, a number of novel treatments have improved practice, with many steroid-sparing agents and a reduction in the progression to splenectomy. Although this has improved clinical care, many therapeutic challenges remain. There is no diagnostic test, no biomarkers to direct treatment and few comparative studies to help management decisions. Development of up to date guidelines is difficult with little high-grade evidence...
March 10, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28203342/optimizing-current-and-emerging-therapies-in-multiple-myeloma-a-guide-for-the-hematologist
#2
REVIEW
Shahzad Raza, Rachael A Safyan, Evan Rosenbaum, Alex S Bowman, Suzanne Lentzsch
Multiple myeloma (MM) is the second most common hematologic malignancy. The diagnosis of MM requires ⩾10% clonal plasma cells in the bone marrow or biopsy-proven plasmacytoma, plus evidence of end-organ damage (hypercalcemia, renal failure, anemia, and lytic bone lesions). The definition of MM has recently been expanded to include a ⩾60% clonal plasma cell burden in the bone marrow, serum involved/uninvolved light chain ratio of ⩾100, or more than one focal lesion on magnetic resonance imaging ⩾5 mm in the absence of end-organ damage...
February 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/28216263/new-insights-into-iron-deficiency-and-iron-deficiency-anemia
#3
REVIEW
Clara Camaschella
Recent advances in iron metabolism have stimulated new interest in iron deficiency (ID) and its anemia (IDA), common conditions worldwide. Absolute ID/IDA, i.e. the decrease of total body iron, is easily diagnosed based on decreased levels of serum ferritin and transferrin saturation. Relative lack of iron in specific organs/tissues, and IDA in the context of inflammatory disorders, are diagnosed based on arbitrary cut offs of ferritin and transferrin saturation and/or marker combination (as the soluble transferrin receptor/ferritin index) in an appropriate clinical context...
February 13, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28241237/association-of-testosterone-levels-with-anemia-in-older-men-a-controlled-clinical-trial
#4
Cindy N Roy, Peter J Snyder, Alisa J Stephens-Shields, Andrew S Artz, Shalender Bhasin, Harvey J Cohen, John T Farrar, Thomas M Gill, Bret Zeldow, David Cella, Elizabeth Barrett-Connor, Jane A Cauley, Jill P Crandall, Glenn R Cunningham, Kristine E Ensrud, Cora E Lewis, Alvin M Matsumoto, Mark E Molitch, Marco Pahor, Ronald S Swerdloff, Denise Cifelli, Xiaoling Hou, Susan M Resnick, Jeremy D Walston, Stephen Anton, Shehzad Basaria, Susan J Diem, Christina Wang, Stanley L Schrier, Susan S Ellenberg
Importance: In one-third of older men with anemia, no recognized cause can be found. Objective: To determine if testosterone treatment of men 65 years or older with unequivocally low testosterone levels and unexplained anemia would increase their hemoglobin concentration. Design, Setting, and Participants: A double-blinded, placebo-controlled trial with treatment allocation by minimization using 788 men 65 years or older who have average testosterone levels of less than 275 ng/dL...
February 21, 2017: JAMA Internal Medicine
https://www.readbyqxmd.com/read/26262795/effects-of-testosterone-administration-for-3-years-on-subclinical-atherosclerosis-progression-in-older-men-with-low-or-low-normal-testosterone-levels-a-randomized-clinical-trial
#5
RANDOMIZED CONTROLLED TRIAL
Shehzad Basaria, S Mitchell Harman, Thomas G Travison, Howard Hodis, Panayiotis Tsitouras, Matthew Budoff, Karol M Pencina, Joseph Vita, Connie Dzekov, Norman A Mazer, Andrea D Coviello, Philip E Knapp, Kathleen Hally, Emma Pinjic, Mingzhu Yan, Thomas W Storer, Shalender Bhasin
IMPORTANCE: Testosterone use in older men is increasing, but its long-term effects on progression of atherosclerosis are unknown. OBJECTIVE: To determine the effect of testosterone administration on subclinical atherosclerosis progression in older men with low or low-normal testosterone levels. DESIGN, SETTING, AND PARTICIPANTS: Testosterone's Effects on Atherosclerosis Progression in Aging Men (TEAAM) was a placebo-controlled, double-blind, parallel-group randomized trial involving 308 men 60 years or older with low or low-normal testosterone levels (100-400 ng/dL; free testosterone <50 pg/mL), recruited at 3 US centers...
August 11, 2015: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/26783877/trends-in-hematological-cancer-in-the-elderly-in-denmark-1980-2012
#6
Lukas F Ocias, Thomas S Larsen, Hanne Vestergaard, Lone S Friis, Niels Abildgaard, Henrik Frederiksen
BACKGROUND: The number of hematological malignancies is expected to increase as the Danish population ages within the next few decades. Despite this, data on the course of hematological cancers among the oldest patients are sparse with many intervention studies focusing on younger age groups. The aim of this study is to present Danish incidence and mortality rates among older patients with non-Hodgkin lymphomas (NHL), multiple myeloma (MM), chronic lymphocytic leukemia (CLL), and acute myeloid leukemia (AML)...
2016: Acta Oncologica
https://www.readbyqxmd.com/read/23869084/long-term-remission-of-t-cell-large-granular-lymphocyte-leukemia-associated-with-rheumatoid-arthritis-after-rituximab-therapy
#7
Divi Cornec, Valérie Devauchelle-Pensec, Sandrine Jousse-Joulin, Thierry Marhadour, Valérie Ugo, Christian Berthou, Nathalie Douet-Guilbert, Alain Saraux
T-cell large granular lymphocyte leukemia (LGLL) is a rare clonal disease often associated with rheumatoid arthritis (RA) and manifests chiefly as neutropenia and recurrent infections. Immunosuppressive agents are the mainstay of treatment, but long-term remissions are rare. We report 2 cases of LGLL in patients with RA successfully treated with rituximab, a monoclonal antibody specific of B cells and approved for treating RA. The first patient experienced a complete LGLL remission that was sustained during the 8-year follow-up after the first rituximab infusion...
August 29, 2013: Blood
https://www.readbyqxmd.com/read/20378561/analysis-of-a-french-cohort-of-patients-with-large-granular-lymphocyte-leukemia-a-report-on-229-cases
#8
Benoît Bareau, Jérôme Rey, Mohamed Hamidou, Jean Donadieu, Jeff Morcet, Oumedaly Reman, Nicolas Schleinitz, Olivier Tournilhac, Mikael Roussel, Thierry Fest, Thierry Lamy
BACKGROUND: Large granular lymphocyte leukemia is a rare lymphoproliferative disorder associated with autoimmune diseases and impaired hematopoiesis. This study describes the clinical and biological characteristics of 229 patients with T-cell or NK-cell large granular lymphocyte leukemia. DESIGN AND METHODS: The diagnosis was based on a large granular lymphocyte expansion (> 0.5x10(9)/L) lasting more than 6 months. Monoclonal T-cell receptor gamma gene rearrangement was detected in all the cases of T-cell large granular lymphocyte leukemia...
September 2010: Haematologica
https://www.readbyqxmd.com/read/25281507/the-analysis-of-clonal-diversity-and-therapy-responses-using-stat3-mutations-as-a-molecular-marker-in-large-granular-lymphocytic-leukemia
#9
Hanna L M Rajala, Thomas Olson, Michael J Clemente, Sonja Lagström, Pekka Ellonen, Tuija Lundan, David E Hamm, Syed Arshi Uz Zaman, Jesus M Lopez Marti, Emma I Andersson, Andres Jerez, Kimmo Porkka, Jaroslaw P Maciejewski, Thomas P Loughran, Satu Mustjoki
T-cell large granular lymphocytic leukemia and chronic lymphoproliferative disorder of natural killer cells are intriguing entities between benign and malignant lymphoproliferation. The molecular pathogenesis has partly been uncovered by the recent discovery of somatic activating STAT3 and STAT5b mutations. Here we show that 43% (75/174) of patients with T-cell large granular lymphocytic leukemia and 18% (7/39) with chronic lymphoproliferative disorder of natural killer cells harbor STAT3 mutations when analyzed by quantitative deep amplicon sequencing...
January 2015: Haematologica
https://www.readbyqxmd.com/read/24679833/the-pathogenesis-and-treatment-of-large-granular-lymphocyte-leukemia
#10
REVIEW
Steven Nathaniel Steinway, Francis LeBlanc, Thomas P Loughran
Large granular lymphocyte (LGL) leukemia is a spectrum of rare lymphoproliferative diseases of T lymphocytes and natural killer cells. These diseases frequently present with splenomegaly, neutropenia, and autoimmune diseases like rheumatoid arthritis. LGL leukemia is more commonly of a chronic, indolent nature; however, rarely, they have an aggressive course. LGL leukemia is thought to arise from chronic antigen stimulation, which drives long-term cell survival through the activation of survival signaling pathways and suppression of pro-apoptotic signals...
May 2014: Blood Reviews
https://www.readbyqxmd.com/read/28039189/factor-xi-deficiency-is-associated-with-decreased-risk-for-cardiovascular-and-venous-thromboembolism-events
#11
Meir Preis, Julianna Hirsch, Antonio Kotler, Ahmad Zoabi, Nili Stein, Gad Rennert, Walid Saliba
Factor XI deficiency is one of the rare inherited coagulation factor deficiencies. However, it incidence is high among Ashkenazi Jewish. Because factor XI displays both procoagulant and antifibrinolytic activities, it has been postulated that an underlying cardiovascular benefit may exist with factor XI deficiency. This historical cohort study was performed using the electronic database of Clalit Health Services, the largest health care provider in Israel. All adults tested for Factor XI activity between 2002 and 2014 were included in the study...
December 30, 2016: Blood
https://www.readbyqxmd.com/read/27870387/primary-myelofibrosis-2017-update-on-diagnosis-risk-stratification-and-management
#12
Ayalew Tefferi
Disease overview: Primary myelofibrosis (PMF) is a myeloproliferative neoplasm (MPN) characterized by stem cell-derived clonal myeloproliferation that is often but not always accompanied by JAK2, CALR or MPL mutation, abnormal cytokine expression, bone marrow fibrosis, anemia, splenomegaly, extramedullary hematopoiesis (EMH), constitutional symptoms, cachexia, leukemic progression and shortened survival. DIAGNOSIS: Diagnosis is based on bone marrow morphology. The presence of JAK2, CALR or MPL mutation is supportive but not essential for diagnosis; approximately 90% of patients carry one of these mutations and 10% are "triple-negative...
December 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27991718/polycythemia-vera-and-essential-thrombocythemia-2017-update-on-diagnosis-risk-stratification-and-management
#13
Ayalew Tefferi, Tiziano Barbui
DISEASE OVERVIEW: Polycythemia Vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation. DIAGNOSIS: PV is defined by a JAK2 mutation, whose absence, combined with normal or increased serum erythropoietin level, makes the diagnosis unlikely...
January 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27797129/alternative-agents-to-prophylactic-platelet-transfusion-for-preventing-bleeding-in-people-with-thrombocytopenia-due-to-chronic-bone-marrow-failure-a-meta-analysis-and-systematic-review
#14
REVIEW
Michael Desborough, Andreas V Hadjinicolaou, Anna Chaimani, Marialena Trivella, Paresh Vyas, Carolyn Doree, Sally Hopewell, Simon J Stanworth, Lise J Estcourt
BACKGROUND: People with thrombocytopenia due to bone marrow failure are vulnerable to bleeding. Platelet transfusions have limited efficacy in this setting and alternative agents that could replace, or reduce platelet transfusion, and are effective at reducing bleeding are needed. OBJECTIVES: To compare the relative efficacy of different interventions for patients with thrombocytopenia due to chronic bone marrow failure and to derive a hierarchy of potential alternative treatments to platelet transfusions...
October 31, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27863761/iron-deficiency-in-cancer-patients
#15
Flávio Augusto Naoum
Anemia is a frequent complication in cancer patients, both at diagnosis and during treatment, with a multifactorial etiology in most cases. Iron deficiency is among the most common causes of anemia in this setting and can develop in nearly half of patients with solid tumors and hematologic malignancies. Surprisingly, this fact is usually neglected by the attending physician in a way that proper and prompt investigation of the iron status is either not performed or postponed. In cancer patients, functional iron deficiency is the predominant mechanism, in which iron availability is reduced due to disease or the therapy-related inflammatory process...
October 2016: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/27768093/the-role-of-maintenance-therapy-in-multiple-myeloma
#16
REVIEW
B Lipe, R Vukas, J Mikhael
Multiple myeloma is the second most common type of blood cancer and remains incurable despite advances in therapy. Current therapy for multiple myeloma includes a phased-approach, often consisting of initial induction therapy, consolidation and maintenance therapy. With an ever-growing landscape of treatment options, the approach to optimal therapy has become increasingly complex. Specifically, controversy surrounds the optimal use and duration of maintenance therapy. We conducted a comprehensive literature search to analyze the most current literature and to provide recommendations for maintenance therapy in multiple myeloma...
October 21, 2016: Blood Cancer Journal
https://www.readbyqxmd.com/read/27737845/cll-an-acquired-immunodeficiency-disease
#17
Clive S Zent
No abstract text is available yet for this article.
October 13, 2016: Blood
https://www.readbyqxmd.com/read/25319751/unveiling-skeletal-fragility-in-patients-diagnosed-with-mgus-no-longer-a-condition-of-undetermined-significance
#18
REVIEW
Matthew T Drake
Monoclonal gammopathy of undetermined significance (MGUS) is a common finding in clinical practice, affecting greater than 3% of adults aged 50 years and older. As originally described, the term MGUS reflected the inherent clinical uncertainty of distinguishing patients with a benign stable monoclonal plasma cell disorder from subjects destined to progress to malignancy. There is now clear epidemiologic evidence, however, that patients with MGUS suffer from a significantly increased fracture risk and that the prevalence of MGUS is increased in patients with osteoporosis...
December 2014: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/21441462/monoclonal-gammopathy-of-undetermined-significance-mgus-and-smoldering-multiple-myeloma-smm-novel-biological-insights-and-development-of-early-treatment-strategies
#19
REVIEW
Neha Korde, Sigurdur Y Kristinsson, Ola Landgren
Monoclonal gammopathy of unknown significance (MGUS) and smoldering multiple myeloma (SMM) are asymptomatic plasma cell dyscrasias, with a propensity to progress to symptomatic MM. In recent years there have been improvements in risk stratification models (involving molecular markers) of both disorders, which have led to better understanding of the biology and probability of progression of MGUS and SMM. In the context of numerous molecular events and heterogeneous risk of progression, developing individualized risk profiles for patients with MGUS and SMM represents an ongoing challenge that has to be addressed by prospective clinical monitoring and extensive correlative science...
May 26, 2011: Blood
https://www.readbyqxmd.com/read/24670051/identification-of-the-potential-risk-factors-for-monoclonal-gammopathy-of-undetermined-significance-of-progression
#20
REVIEW
Rong Li, Juan Du, Jian Hou
BACKGROUNDS: Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant plasma cell disorder. The etiology of MGUS progression remains unclear and is a current topic of investigation. METHODS: This review summarizes the essential features of MGUS and the potential risk factors for MGUS of progression. RESULTS: Many clinical studies have been conducted to identify the critical risk markers that play important roles in progression...
January 2015: Hematology (Amsterdam, Netherlands)
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