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Hematology

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By Ronald Lands Benign hematologist
https://www.readbyqxmd.com/read/29110361/hairy-cell-leukemia-2018-update-on-diagnosis-risk-stratification-and-treatment
#1
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
#2
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
#3
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
#4
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
#5
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...
October 19, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28115367/lgl-leukemia-from-pathogenesis-to-treatment
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
https://www.readbyqxmd.com/read/28655976/iron-deficiency-anemia-in-chronic-liver-disease-etiopathogenesis-diagnosis-and-treatment
#16
REVIEW
Eleana Gkamprela, Melanie Deutsch, Dimitrios Pectasides
Chronic liver disease is accompanied by multiple hematological abnormalities. Iron deficiency anemia is a frequent complication of advanced liver disease. The etiology is multifactorial, mostly due to chronic hemorrhage into the gastrointestinal tract. The diagnosis of iron deficiency anemia is very challenging, as simple laboratory methods, including serum iron, ferritin, transferrin saturation (Tsat), and mean corpuscular volume are affected by the liver disease itself or the cause of the disease, resulting in difficulty in the interpretation of the results...
2017: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/22367928/hitlights-a-career-perspective-on-heparin-induced-thrombocytopenia
#17
REVIEW
Theodore E Warkentin
Two decades of research into heparin-induced thrombocytopenia (HIT) permit a personal historical perspective on this fascinating syndrome. Previously, the frequency of HIT was unknown, although complicating thrombosis was believed to be rare and primarily arterial. The opportunity to apply a remarkable test for "HIT antibodies"--the (14) C-serotonin-release assay (SRA)--to serial plasma samples obtained during a clinical trial of heparin thromboprophylaxis, provided insights into the peculiar nature of HIT, such as, its prothrombotic nature--including its strong association with venous thrombosis (RR = 11...
May 2012: American Journal of Hematology
https://www.readbyqxmd.com/read/28615223/daratumumab-yields-rapid-and-deep-hematologic-responses-in-patients-with-heavily-pretreated-al-amyloidosis
#18
Gregory P Kaufman, Stanley L Schrier, Richard A Lafayette, Sally Arai, Ronald M Witteles, Michaela Liedtke
The majority of patients with immunoglobulin light chain amyloidosis (AL) fail to achieve a complete response (CR) to standard light chain suppressive chemotherapy, and almost all patients eventually experience hematologic relapse and progression of organ involvement. Additional well-tolerated treatment options are needed. We present our retrospective experience of 25 consecutive previously treated AL patients who received daratumumab, a CD38-directed monoclonal antibody approved for the treatment of multiple myeloma...
August 17, 2017: Blood
https://www.readbyqxmd.com/read/28483766/how-i-treat-recurrent-venous-thromboembolism-in-patients-receiving-anticoagulant-therapy
#19
REVIEW
Sam Schulman
Oral anticoagulant therapy for venous thromboembolism is very effective. When oral anticoagulants are managed well, the risk of recurrence is approximately 2 per 100 patient-years. The main reasons for a breakthrough event are underlying disease and subtherapeutic drug levels. The most common underlying disease that results in recurrence on treatment is cancer. Subtherapeutic drug levels can be caused by poor adherence to the drug regimen, interactions with other drugs or food, or inappropriate dosing. It is important to investigate and understand the cause whenever such an event occurs and to improve management of anticoagulants thereby avoiding further recurrences...
June 22, 2017: Blood
https://www.readbyqxmd.com/read/28416508/hus-and-atypical-hus
#20
REVIEW
T Sakari Jokiranta
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by Shiga toxin-producing Escherichia coli (STEC) infection, as atypical HUS (aHUS), usually caused by uncontrolled complement activation, or as secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection with STEC, whereas aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation...
May 25, 2017: Blood
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