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Hematology

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By Ronald Lands Benign hematologist
https://www.readbyqxmd.com/read/29295846/splenectomy-for-immune-thrombocytopenia-down-but-not-out
#1
Shruti Chaturvedi, Donald M/ Arnold, Keith R McCrae
Splenectomy is an effective therapy for steroid-refractory or dependent immune thrombocytopenia (ITP). With the advent of medical alternatives such as rituximab and thrombopoietin receptor antagonists (TPO-RA), the use of splenectomy has declined and is generally reserved for patients that fail multiple medical therapies. Splenectomy removes the primary site of platelet clearance and autoantibody production, and offers the highest rate of durable response (50-70%) compared to other ITP therapies. However, there are no reliable predictors of splenectomy response, and long-term risks of infection and cardiovascular complications must be considered...
January 2, 2018: Blood
https://www.readbyqxmd.com/read/28088988/clonal-hematopoiesis
#2
REVIEW
Max Jan, Benjamin L Ebert, Siddhartha Jaiswal
Cancer results from multistep pathogenesis, yet the pre-malignant states that precede the development of many hematologic malignancies have been difficult to identify. Recent genomic studies of blood DNA from tens of thousands of people have revealed the presence of remarkably common, age-associated somatic mutations in genes associated with hematologic malignancies. These somatic mutations drive the expansion from a single founding cell to a detectable hematopoietic clone. Owing to the admixed nature of blood that provides a sampling of blood cell production throughout the body, clonal hematopoiesis is a rare view into the biology of pre-malignancy and the direct effects of pre-cancerous lesions on organ dysfunction...
January 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/29231133/daratumumab-plus-bortezomib-melphalan-and-prednisone-for-untreated-myeloma
#3
María-Victoria Mateos, Meletios A Dimopoulos, Michele Cavo, Kenshi Suzuki, Andrzej Jakubowiak, Stefan Knop, Chantal Doyen, Paulo Lucio, Zsolt Nagy, Polina Kaplan, Ludek Pour, Mark Cook, Sebastian Grosicki, Andre Crepaldi, Anna M Liberati, Philip Campbell, Tatiana Shelekhova, Sung-Soo Yoon, Genadi Iosava, Tomoaki Fujisaki, Mamta Garg, Christopher Chiu, Jianping Wang, Robin Carson, Wendy Crist, William Deraedt, Huong Nguyen, Ming Qi, Jesus San-Miguel
Background The combination of bortezomib, melphalan, and prednisone is a standard treatment for patients with newly diagnosed multiple myeloma who are ineligible for autologous stem-cell transplantation. Daratumumab has shown efficacy in combination with standard-of-care regimens in patients with relapsed or refractory multiple myeloma. Methods In this phase 3 trial, we randomly assigned 706 patients with newly diagnosed multiple myeloma who were ineligible for stem-cell transplantation to receive nine cycles of bortezomib, melphalan, and prednisone either alone (control group) or with daratumumab (daratumumab group) until disease progression...
December 12, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29214694/myelodysplastic-syndromes-2018-update-on-diagnosis-risk-stratification-and-management
#4
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
https://www.readbyqxmd.com/read/29183887/how-i-manage-monoclonal-gammopathy-of-undetermined-significance
#5
Ronald S Go, S Vincent Rajkumar
Monoclonal gammopathy of undetermined significance (MGUS) is, in many ways, a unique hematologic entity. Unlike most hematologic conditions in which the diagnosis is intentional and credited to hematologists, the discovery of MGUS is most often incidental and made by non-hematologists. MGUS is considered an obligate precursor to several lymphoplasmacytic malignancies including immunoglobulin light chain amyloidosis, multiple myeloma, and Waldenström macroglobulinemia. Therefore, long-term follow-up is generally recommended...
November 28, 2017: Blood
https://www.readbyqxmd.com/read/29110361/hairy-cell-leukemia-2018-update-on-diagnosis-risk-stratification-and-treatment
#6
Xavier Troussard, Edouard Cornet
DISEASE OVERVIEW: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders, characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment. DIAGNOSIS: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation...
December 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28854095/porphyria
#7
REVIEW
D Montgomery Bissell, Karl E Anderson, Herbert L Bonkovsky
New England Journal of Medicine, Volume 377, Issue 9, Page 862-872, August 2017.
August 31, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/26574400/car-t-cells-merge-into-the-fast-lane-of-cancer-care
#8
REVIEW
Noelle V Frey, David L Porter
Chimeric antigen receptors (CARs) can be introduced into T-cells redirecting them to target specific tumor antigens. CAR-modified T cells targeting CD19 have shown remarkable activity against CD19+ malignancies including B cell acute lymphocytic leukemia (ALL), chronic lymphocytic leukemia (CLL), and non-Hodgkin lymphomas (NHL). Complete remission rates as high as 90% have been observed for patients with relapsed and refractory ALL and greater than 50% response rates have been seen in heavily pre-treated CLL and NHL...
January 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/25461996/chronic-lymphocytic-leukemia-a-clinical-review
#9
REVIEW
Chadi Nabhan, Steven T Rosen
IMPORTANCE: The most common leukemia is chronic lymphocytic leukemia (CLL). Every year, there are 15 000 new diagnoses and 5000 CLL deaths in the United States. Although therapeutic choices were once limited, treatment of this disease has vastly improved in the last decade. OBJECTIVE: Evidence-based review of the diagnosis, staging, and treatment of CLL. EVIDENCE REVIEW: PubMed, Cochrane Library, Scopus, and Google Scholar databases were searched through August 28, 2014...
December 3, 2014: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29048128/how-i-manage-relapse-of-chronic-myeloid-leukaemia-after-stopping-tyrosine-kinase-inhibitor-therapy
#10
REVIEW
Delphine Rea, François-Xavier Mahon
During the last 10 years, clinical trials formally demonstrated that about 50% of patients with chronic phase (CP) chronic myeloid leukaemia (CML) who achieve and maintain deep molecular responses for a prolonged period of time during treatment with imatinib or new generation tyrosine kinase inhibitors (TKIs) may successfully stop their anti-leukaemic therapy. Based on the accumulated knowledge from abundant clinical trial experience, TKI discontinuation is becoming an important goal to achieve and is about to enter clinical practice...
January 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/28115367/lgl-leukemia-from-pathogenesis-to-treatment
#11
REVIEW
Thierry Lamy, Aline Moignet, Thomas P Loughran
Large granular lymphocyte (LGL) leukemia has been recognized by the World Health Organization classifications amongst mature T-cell and natural killer (NK) cell neoplasms. There are 3 categories: chronic T-cell leukemia and NK-cell lymphocytosis, which are similarly indolent diseases characterized by cytopenias and autoimmune conditions as opposed to aggressive NK-cell LGL leukemia. Clonal LGL expansion arise from chronic antigenic stimulation, which promotes dysregulation of apoptosis, mainly due to constitutive activation of survival pathways including Jak/Stat, MapK, phosphatidylinositol 3-kinase-Akt, Ras-Raf-1, MEK1/extracellular signal-regulated kinase, sphingolipid, and nuclear factor-κB...
March 2, 2017: Blood
https://www.readbyqxmd.com/read/28971908/comparison-of-up-front-treatments-for-newly-diagnosed-immune-thrombocytopenia-a-systematic-review-and-network-meta-analysis
#12
Yasuyuki Arai, Tomoyasu Jo, Hiroyuki Matsui, Tadakazu Kondo, Akifumi Takaori-Kondo
Corticosteroids such as prednisolone and dexamethasone have been established as up-front therapy for the treatment of newly diagnosed immune thrombocytopenia. Recent studies have indicated that other treatments such rituximab or thrombopoietin receptor agonist can also be effective choices. We performed a systematic review and network meta-analysis to establish a clinically meaningful hierarchy of the efficacy and safety of treatment for newly diagnosed primary immune thrombocytopenia in adults. Randomized controlled trials evaluating medical treatments for newly diagnosed immune thrombocytopenia were included...
September 29, 2017: Haematologica
https://www.readbyqxmd.com/read/28930509/thrombophilia-testing-and-venous-thrombosis
#13
REVIEW
Jean M Connors
New England Journal of Medicine, Volume 377, Issue 12, Page 1177-1187, September 2017.
September 21, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28829751/the-older-the-faster-aged-neutrophils-in-inflammation
#14
COMMENT
Elzbieta Kolaczkowska
No abstract text is available yet for this article.
November 10, 2016: Blood
https://www.readbyqxmd.com/read/28809726/clinical-and-laboratory-associations-with-persistent-hyperferritinemia-in-373-black-hemochromatosis-and-iron-overload-screening-study-participants
#15
James C Barton, J Clayborn Barton, Paul C Adams
BACKGROUND: 373 black participants had elevated screening and post-screening serum ferritin (SF) (> 300 μg/L men; > 200 μg/L women). MATERIAL AND METHODS: We retrospectively studied SF and post-screening age; sex; body mass index; transferrin saturation (TS); ALT; AST; GGT; elevated C-reactive protein; ß-thalassemia; neutrophils; lymphocytes; monocytes; platelets; metacarpophalangeal joint hypertrophy; hepatomegaly; splenomegaly; diabetes; HFE H63D positivity; iron/alcohol intakes; and blood/erythrocyte transfusion units...
September 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/26209894/eosinophilia-in-hematologic-disorders
#16
REVIEW
Lorenzo Falchi, Srdan Verstovsek
Eosinophilia in the peripheral blood can be the manifestation various medical conditions, including benign or malignant disorders. There are 3 main types of eosinophilia-associated myeloid neoplasms (MN-eos): myeloid and lymphoid neoplasms, chronic eosinophilic leukemia not otherwise specified, and idiopathic hypereosinophilic syndrome (HES). Imatinib mesylate has revolutionized the treatment of molecularly defined MN-eos, and novel agents have been successfully used to treat HES. The discovery of new, recurrent molecular alterations in patients with MN-eos may improve their diagnosis and therapy...
August 2015: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28028030/myeloid-neoplasms-with-eosinophilia
#17
REVIEW
Andreas Reiter, Jason Gotlib
Molecular diagnostics has generated substantial dividends in dissecting the genetic basis of myeloid neoplasms with eosinophilia. The family of diseases generated by dysregulated fusion tyrosine kinase (TK) genes is recognized by the World Health Organization (WHO) category, "Myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB, or FGFR1, or with PCM1-JAK2" In addition to myeloproliferative neoplasms (MPN), these patients can present with myelodysplastic syndrome/MPN, as well as de novo or secondary mixed-phenotype leukemias or lymphomas...
February 9, 2017: Blood
https://www.readbyqxmd.com/read/28680366/fatal-pulmonary-embolism-following-splenectomy-in-a-patient-with-evan-s-syndrome-case-report-and-review-of-the-literature
#18
Varun Monga, Seth M Maliske, Usha Perepu
BACKGROUND: Evans syndrome (ES) is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Splenectomy is one of the treatment options for disease refractory to medical therapy. Venous thromboembolism (VTE) following splenectomy for hematological diseases has an incidence of 10%. CASE PRESENTATION: Here we describe a case report of a young patient hospitalized with severe hemolytic anemia with Hgb 4...
2017: Thrombosis Journal
https://www.readbyqxmd.com/read/28741899/what-the-neighbors-say
#19
EDITORIAL
(no author information available yet)
No abstract text is available yet for this article.
August 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28662310/thrombotic-thrombocytopenic-purpura-pathogenesis-diagnosis-and-potential-novel-therapeutics
#20
REVIEW
M Saha, J K McDaniel, X L Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal clinical syndrome, is primarily caused by autoantibodies against the von Willebrand factor (VWF)-cleaving metalloprotease ADAMTS-13. In general, severe deficiency of plasma ADAMTS-13 activity (< 10 IU dL-1 ) with or without detectable inhibitory autoantibodies against ADAMTS-13 supports the diagnosis of TTP. A patient usually presents with thrombocytopenia and microangiopathic hemolytic anemia (i.e. schistocytes, elevated serum lactate dehydrogenase, decreased hemoglobin and haptoglobin) without other known etiologies that cause thrombotic microangiopathy (TMA)...
October 2017: Journal of Thrombosis and Haemostasis: JTH
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