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By Javier Pilcante Hematologist
Rebecca L Larsen, Natasha M Savage
Eosinophilia is typically secondary, that is, reactive, in nature and is associated with a wide variety of neoplastic and non-neoplastic disorders. Clonal eosinophilia is also seen in a wide variety of hematopoietic neoplasms, and sub-classification can be diagnostically challenging. A proper evaluation of persistent eosinophilia involves correlation of clinical history, laboratory data, cellular morphology, and ancillary testing. Knowledge of appropriate ancillary testing is necessary for a timely diagnosis...
November 30, 2018: International Journal of Laboratory Hematology
Noémie Kraaijpoel, Marc Carrier
Venous thromboembolism (VTE), which includes deep vein thrombosis and pulmonary embolism, is a common complication of cancer and is associated with significant morbidity and mortality. Several cancer-related risk factors contribute to the development of VTE including cancer type and stage, chemotherapy, surgery, and patient-related factors such as advanced age and immobilization. Patients with cancer frequently undergo diagnostic imaging scans for cancer staging and treatment response evaluation, which is increasing the underlying risk of VTE detection...
January 24, 2019: Blood
Guillermo Montalban-Bravo, Guillermo Garcia-Manero
DISEASE OVERVIEW: The myelodysplastic syndromes (MDS) are a very heterogeneous group of myeloid disorders characterized by peripheral blood cytopenias and increased risk of transformation to acute myelogenous leukemia (AML). MDS occurs more frequently in older males and in individuals with prior exposure to cytotoxic therapy. DIAGNOSIS: Diagnosis of MDS is based on morphological evidence of dysplasia upon visual examination of a bone marrow aspirate and biopsy. Information obtained from additional studies such as karyotype, flow cytometry or molecular genetics is usually complementary and may help refine diagnosis...
January 2018: American Journal of Hematology
Morie A Gertz
DISEASE OVERVIEW: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity. DIAGNOSIS: Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in >90% of patients and is found in the majority of IgM monoclonal gammopathy of undetermined significance patients...
October 17, 2018: American Journal of Hematology
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No abstract text is available yet for this article.
August 16, 2018: American Journal of Hematology
Elihu H Estey
Outcome in patients with acute myeloid leukemia (AML) ranges from death within a few days of beginning treatment (treatment related mortality, TRM) to likely cure. The major reason patients are not cured is resistance to treatment, often manifested as relapse from remission, rather than, even in older patients, TRM, whose incidence is decreasing. Knowledge of the pre-treatment mutation status of various genes has improved our ability to assign initial treatment and, of particular importance, knowledge of whether patients ostensibly in remission have measurable residual disease should influence subsequent management...
October 2018: American Journal of Hematology
Prajwal Chaitanya Boddu, Tapan Mahendra Kadia
The application of next generation sequencing (NGS) has enhanced our understanding of the genetic landscape in acquired aplastic anemia (AA). Parallel progress has been in addressing aspects underlying immune dysregulation in disease pathogenesis. Novel insights into the molecular and biologic mechanisms have led to a shift in the paradigm of AA, from a solely autoimmune pathogenic concept toward its recognition as a multifaceted pathophysiology characterized by cytogenetic abnormalities, recurrent somatic mutations, telomere attrition, and immune dysregulation...
October 31, 2018: European Journal of Haematology
T C El-Galaly, D Villa, L C Gormsen, J Baech, A Lo, C Y Cheah
FDG-PET/CT is the current state-of-the-art imaging in lymphoma and plays a central role in treatment decisions. At diagnosis, accurate staging is crucial for appropriate therapy selection: FDG-PET/CT can identify areas of lymphoma missed by CT alone and avoid under-treatment of patients with advanced disease stage who would have been misclassified as having limited stage disease by CT. Particularly in Hodgkin lymphoma, positive interim FDG-PET/CT scans are adversely prognostic for clinical outcomes and can inform PET-adapted treatment strategies, but such data are less consistent in diffuse large B-cell lymphoma...
October 2018: Journal of Internal Medicine
Sameer A Parikh
The treatment landscape for patients with chronic lymphocytic leukemia (CLL) has changed considerably with the introduction of very effective oral targeted therapies (such as ibrutinib, idelalisib, and venetoclax), and next-generation anti-CD20 monoclonal antibodies (such as obinutuzumab). These agents lead to improved outcomes in CLL, even among patients with high-risk features, such as del17p13 or TP53 mutation and unmutated immunoglobulin heavy chain (IGHV) genes. Each of these treatments is associated with a unique toxicity profile; in the absence of randomized data, the choice of one type of treatment over another depends on the co-morbidities of the patient...
October 3, 2018: Blood Cancer Journal
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
J J López-Núñez, J Trujillo-Santos, M Monreal
Current guidelines for anticoagulant therapy do not so far suggest any form of differentiated approach to cancer patients with venous thromboembolism (VTE). This review article provides an overview of the published literature in cancer patients with VTE, mostly using data from the RIETE registry. Our findings provide some insights into what factors may be used to guide physicians in adapting recommended anticoagulant regimens to the individual patient, as oncologists are increasingly doing with cancer treatments...
December 2018: Journal of Thrombosis and Haemostasis: JTH
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
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
Alessandro M Vannucchi
Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with JAK2 mutations (V617F or exon 12) in almost all cases. The World Health Organization has defined the criteria for diagnosis, but it is still unclear which parameter (hemoglobin or hematocrit) is the most reliable for demonstrating increased red cell volume and for monitoring response to therapy; also, the role of bone marrow biopsy is being revisited. PV is associated with reduced survival because of cardiovascular complications and progression to post-PV myelofibrosis or leukemia...
November 20, 2014: Blood
Ayalew Tefferi, Paola Guglielmelli, Animesh Pardanani, Alessandro M Vannucchi
Two novel prognostic systems for primary myelofibrosis (PMF) were recently unveiled: GIPSS (genetically inspired prognostic scoring system) and MIPSS70 (mutation-enhanced international prognostic scoring system for transplant-age patients). GIPSS is based exclusively on genetic markers: mutations and karyotype. MIPSS70 includes mutations and clinical risk factors. In its most recent adaptation, the prognostic value of MIPSS70 has been bolstered by the inclusion of a three-tiered cytogenetic risk stratification and use of hemoglobin thresholds that are adjusted for sex and severity (MIPSS70+ version 2...
July 31, 2018: Blood Cancer Journal
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
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
Hanyin Wang, Hande Tuncer
The value of combination therapy for refractory ITP is not well defined. We present the case of a 29-year-old male with severe ITP refractory to initial standard therapy including steroids, IVIG, and subsequent splenectomy, who was treated with the combination therapy of rituximab, romiplostim, and mycophenolate and eventually developed thrombocytosis requiring plateletpheresis. Our case highlights the importance of the need to understand predictors of response to standard upfront treatment of acute ITP.
2018: Case Reports in Hematology
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
Elias Jabbour, Hagop Kantarjian
DISEASE OVERVIEW: Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm with an incidence of 1-2 cases per 100 000 adults. It accounts for approximately 15% of newly diagnosed cases of leukemia in adults. DIAGNOSIS: CML is characterized by a balanced genetic translocation, t(9;22)(q34;q11.2), involving a fusion of the Abelson gene (ABL1) from chromosome 9q34 with the breakpoint cluster region (BCR) gene on chromosome 22q11.2. This rearrangement is known as the Philadelphia chromosome...
March 2018: American Journal of Hematology
2018-07-24 23:47:16
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