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Hematology—the Education Program of the American Society of Hematology

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https://www.readbyqxmd.com/read/26637785/evens-am-kostakoglu-l-the-role-of-fdg-pet-in-defining-prognosis-of-hodgkin-lymphoma-for-early-stage-disease-hematology-am-soc-hematol-educ-program-2014-2014-135-143
#1
(no author information available yet)
No abstract text is available yet for this article.
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637784/evidence-for-janus-kinase-jak-inhibitors-for-the-prevention-of-major-morbid-events-in-patients-with-myeloproliferative-neoplasms-mpns
#2
Naveen Pemmaraju, Ruben Mesa
A 69-year-old man presents with a Dynamic International Prognostic Scoring System (DIPSS) intermediate-risk 2 post polycythemia vera myelofibrosis with significant splenomegaly, 30 pound weight loss, constitutional symptoms, and 2% peripheral blood blasts. He has no other significant past medical history and no other major comorbid conditions. You are asked by the hematology fellow you are supervising whether or not treating this patient with a Janus kinase (JAK) inhibitor will decrease major morbid events.
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637783/syndromes-of-thrombotic-microangiopathy-associated-with-pregnancy
#3
James N George, Carla M Nester, Jennifer J McIntosh
When a pregnant or postpartum woman presents with sudden and severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia, three syndromes that require urgent care must be considered: (1) preeclampsia with severe features/hemolysis, elevated liver function tests, low platelets (PE/HELLP) syndrome; (2) thrombotic thrombocytopenic purpura (TTP); and (3) complement-mediated thrombotic microangiopathy (C-TMA; also referred to as atypical hemolytic-uremic syndrome). The distinction among these three syndromes is often unclear because they share multiple clinical features...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637782/treatment-of-thrombotic-thrombocytopenic-purpura-beyond-therapeutic-plasma-exchange
#4
REVIEW
Paul Coppo, Antoine Froissart
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of 80%-85%. However, relapses occur in ~40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637781/what-s-new-in-the-diagnosis-and-pathophysiology-of-thrombotic-thrombocytopenic-purpura
#5
J Evan Sadler
Severe ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency causes thrombotic thrombocytopenic purpura (TTP), which is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and the absence of oliguric or anuric renal failure. However, some patients with this constellation of findings do not have ADAMTS13 deficiency, and some patients with ADAMTS13 deficiency have renal failure or relatively normal blood counts. Consequently, many investigators and clinicians have incorporated severe ADAMTS13 deficiency into the case definition of TTP...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637780/treatment-of-patients-with-transformed-lymphoma
#6
REVIEW
Silvia Montoto
Histologic transformation (HT) is a frequent event in the clinical course of patients with indolent lymphoma. Most of the available data in the literature comes from studies on transformation of follicular lymphoma (FL), as this is the most common indolent lymphoma; however, HT is also well documented following small lymphocytic lymphoma/chronic lymphocytic leukaemia (SLL/CLL), lymphoplasmacytic lymphoma (LPL), or marginal zone lymphoma (MZL), amongst other types of lymphoma, albeit most of the studies on transformation in these subtypes are case reports or short series...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637779/using-the-pathology-report-in-initial-treatment-decisions-for-diffuse-large-b-cell-lymphoma-time-for-a-precision-medicine-approach
#7
REVIEW
Jonathan W Friedberg
Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non Hodgkin lymphoma in the Western world, and is potentially curable with standard R-CHOP chemoimmunotherapy. Historically, clinical risk assessments provided prognostic information, but did not define treatment approach. We are now in an era where the heterogeneity of DLBCL is defined genetically and molecularly, and rational subset-specific therapeutic targets are guiding clinical trials. Primary mediastinal DLBCL is a unique clinicopathologic entity, and alternatives to R-CHOP may confer superior outcome...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637778/update-in-large-cell-lymphoma-understanding-the-pathology-report
#8
REVIEW
Eric D Hsi
The diffuse aggressive large B-cell lymphomas are a heterogeneous group of B-cell malignancies. Although many are readily recognized due to characteristic clinical and pathologic features, several problematic areas still exist in diagnosis of these lymphomas due to a variety of reasons that include imprecise or difficult-to-apply diagnostic criteria, gaps in our understanding of lymphoma biology, and limitations in technologies available in the clinical laboratory compared to the research laboratory. This may result in some degree of confusion in the pathology report, particularly if the issues are not clearly explained, leading to frustration or misinterpretation on the part of the reader...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637777/new-approaches-to-transplantation-in-acute-myelogenous-leukemia
#9
REVIEW
Marcos de Lima
Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative therapeutic option for acute myelogenous leukemia (AML). This is due to the combined effect of chemo/radiation therapy and the immunologic graft-versus-leukemia effect. The field of HSCT has benefited from advances in a variety of "fronts," including our increasing ability to break the human leukocyte antigen barrier, which has led to greater access to transplantation. Furthermore, progress in the biologic, genetic, and pharmacologic arenas is creating a scenario where traditional borders between transplant and non-transplant therapies are less clear...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637776/antigen-specific-immunotherapies-for-acute-myeloid-leukemia
#10
REVIEW
Sarah A Buckley, Roland B Walter
Antigen-specific immunotherapies have emerged as important components of curative treatment algorithms for many cancers. In acute myeloid leukemia (AML), success has been less obvious. Nonetheless, among the few drugs shown to improve survival in recent randomized trials is the CD33 antibody-drug conjugate gemtuzumab ozogamicin. Significant antileukemic activity is also well documented for radioimmunoconjugates targeting CD33, CD45, or CD66. These therapeutics can intensify conditioning before hematopoietic cell transplantation, but their effect on patient outcomes needs clarification...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637775/molecularly-targeted-therapies-for-acute-myeloid-leukemia
#11
REVIEW
Eytan M Stein
The past 15 years have seen major leaps in our understanding of the molecular genetic mutations that act as drivers of acute myeloid leukemia (AML). Clinical trials of agents against specific mutant proteins, such as FLT3-internal tandem duplications (ITDs) and isocitrate dehydrogenase mutations (IDHs) are ongoing. This review discusses agents in clinical trials that target specific gene mutations and/or epigenetic targets.
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637774/uncommon-histiocytic-disorders-rosai-dorfman-juvenile-xanthogranuloma-and-erdheim-chester-disease
#12
Julien Haroche, Oussama Abla
Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and Erdheim-Chester disease (ECD) are non-Langerhans cell (non-LCH) disorders arising from either a dendritic or a macrophage cell. RDD is a benign disorder that presents with massive lymphadenopathy, but can have extranodal involvement. In most cases, RDD is self-limited and observation is the standard approach. Treatment is restricted to patients with life-threatening, multiple-relapsing, or autoimmune-associated disease. JXG is a pediatric histiocytosis characterized by xanthomatous skin lesions that usually resolve spontaneously...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637773/treatment-of-langerhans-cell-histiocytosis-role-of-braf-mapk-inhibition
#13
Oussama Abla, Sheila Weitzman
Langerhans cell histiocytosis (LCH) is a clonally derived neoplasm with a highly variable clinical course. Although LCH was once considered a disorder of immune regulation, the identification of activating mutations in the proto-oncogene BRAF-V600E in ~50%-60% of cases and MEK and ERK phosphorylation in 100% of examined cases, has changed the definition of LCH to a dendritic cell neoplasm with a strong inflammatory component. Current international LCH trials are focused on further improving the outcome of high-risk multisystem LCH patients, by decreasing the reactivation rate, optimizing early salvage regimens, and preventing late sequelae...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637772/biological-and-clinical-significance-of-somatic-mutations-in-langerhans-cell-histiocytosis-and-related-histiocytic-neoplastic-disorders
#14
REVIEW
Carl E Allen, D Williams Parsons
Langerhans cell histiocytosis (LCH), juvenile xanthogranuloma (JXG), and Erdheim-Chester disease (ECD) represent histiocytic disorders with a wide range of clinical manifestations. Until recently, mechanisms of pathogenesis have been speculative and debate has focused on classification of these conditions as reactive versus neoplastic. Genomic studies have been challenged by scarce tissue specimens, as well as heterogeneous nature of the lesions with variable infiltration of pathologic histiocytes. Whole-exome sequencing recently revealed a very low frequency of somatic mutations in LCH, JXG, and ECD compared to other neoplastic disorders...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637771/peripheral-t-cell-lymphoma-nos-and-anaplastic-large-cell-lymphoma
#15
Anne W Beaven, Louis F Diehl
Peripheral T-cell lymphomas (PTCL), with the exception of anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL), have a very poor prognosis. Although current first line chemotherapy continues to be a CHOP-like (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen there is now data suggesting that the addition of etoposide in younger patients improves outcomes. Even for those patients who do have a response to therapy, the risk of relapse remains quite high. Although autologous transplant in first remission is often used, its role as consolidation therapy in first remission remains unclear and may preferentially benefit low-risk patients...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637770/zebras-and-hen-s-teeth-recognition-and-management-of-rare-t-and-nk-lymphomas
#16
Neha Mehta-Shah, Steven Horwitz
Although all the peripheral T-cell lymphomas are uncommon, there are some entities that are truly rare. Subtypes, such as enteropathy-associated T-cell lymphoma, hepatosplenic T-cell lymphoma, extranodal NK/T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma, have an approximate annual incidence in United States of <500 each. In these very rare subtypes, there is limited data to guide clinical decision-making. As such, our treatment decisions are often based on extrapolation, case series, personal experience, and biases...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637769/choosing-a-systemic-treatment-for-advanced-stage-cutaneous-t-cell-lymphoma-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#17
Madeleine Duvic
No abstract text is available yet for this article.
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637768/advances-in-therapies-for-non-hodgkin-lymphoma-in-children
#18
REVIEW
Rachel Kobos, William Terry
Pediatric patients with newly diagnosed, non-Hodgkin Lymphoma (NHL) have an excellent overall survival. However, therapy regimens are associated with acute toxicity and late effects. Furthermore, patients with relapsed or refractory disease have relatively few options with proven clinical benefit. Both histologic and molecular differences exist between adult and pediatric NHL preventing simple translation of adult NHL successes into improvements in pediatric NHL treatment. This review summarizes the introduction of targeted therapies into frontline treatments for patients with anaplastic large-cell lymphoma and CD20-positive tumors, with the goal of improving overall survival while limiting both short- and long-term toxicities...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637767/hodgkin-lymphoma-in-children-and-adolescents-improving-the-therapeutic-index
#19
REVIEW
Kara M Kelly
Hodgkin lymphoma (HL) is a highly curable form of childhood cancer, with estimated 5 year survival rates exceeding 98%. However, the establishment of a "standard of care" approach to its management is complicated by the recognition that long-term overall survival declines in part from delayed effects of therapy and that there continue to be subgroups of patients at risk for relapse for which prognostic criteria cannot adequately define. This challenge has resulted in the development of various strategies aimed at identifying the optimal balance between maintaining overall survival and avoidance of long-term morbidity of therapy, often representing strategies quite different from those used for adults with HL...
2015: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/26637766/acute-myeloid-leukemia-in-children-and-adolescents-identification-of-new-molecular-targets-brings-promise-of-new-therapies
#20
REVIEW
E Anders Kolb, Soheil Meshinchi
Recent reports of recurrent mutations in childhood acute myeloid leukemia (AML) have identified potential targets for new therapeutic strategies. Acute promyelocytic leukemia (APL) is characterized commonly by a fusion between the PML gene and the RARA gene, genes targetable by arsenic (ATO) and retinoic acid (ATRA), respectively. A mutation in GATA1, common in AML of Down syndrome (ML-DS), renders cells more susceptible to cytarabine and anthracyclines, thus permitting targeted dose reductions to preserve high survival rates while reducing toxicity...
2015: Hematology—the Education Program of the American Society of Hematology
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