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Hematology

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https://www.readbyqxmd.com/read/28665419/acute-lymphoblastic-leukemia-a-comprehensive-review-and-2017-update
#1
REVIEW
T Terwilliger, M Abdul-Hay
Acute lymphoblastic leukemia (ALL) is the second most common acute leukemia in adults, with an incidence of over 6500 cases per year in the United States alone. The hallmark of ALL is chromosomal abnormalities and genetic alterations involved in differentiation and proliferation of lymphoid precursor cells. In adults, 75% of cases develop from precursors of the B-cell lineage, with the remainder of cases consisting of malignant T-cell precursors. Traditionally, risk stratification has been based on clinical factors such age, white blood cell count and response to chemotherapy; however, the identification of recurrent genetic alterations has helped refine individual prognosis and guide management...
June 30, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28360039/how-i-treat-autoimmune-hemolytic-anemia
#2
REVIEW
Ronald S Go, Jeffrey L Winters, Neil E Kay
Autoimmune hemolytic anemia (AIHA) is an uncommon entity that presents diagnostic, prognostic, and therapeutic dilemmas despite being a well-recognized entity for over 150 years. This is because of significant differences in the rates of hemolysis and associated diseases and because there is considerable clinical heterogeneity. In addition, there is a lack of clinical trials required to refine and update standardized and evidence-based therapeutic approaches. To aid the clinician in AIHA management, we present four vignettes that represent and highlight distinct clinical presentations with separate diagnostic and therapeutic pathways that we use in our clinical practice setting...
June 1, 2017: Blood
https://www.readbyqxmd.com/read/27766049/management-of-venous-thromboembolism-an-update
#3
REVIEW
Siavash Piran, Sam Schulman
Venous thromboembolism (VTE), which constitutes pulmonary embolism and deep vein thrombosis, is a common disorder associated with significant morbidity and mortality. Landmark trials have shown that direct oral anticoagulants (DOACs) are as effective as conventional anticoagulation with vitamin K antagonists (VKA) in prevention of VTE recurrence and associated with less bleeding. This has paved the way for the recently published guidelines to change their recommendations in favor of DOACs in acute and long-term treatment of VTE in patients without cancer...
2016: Thrombosis Journal
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#4
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27959283/neonates-with-sickle-cell-disease-are-vulnerable-to-blue-light-phototherapy-induced-oxidative-stress-and-proinflammatory-cytokine-elevations
#5
Hemakshi Chaudhari, Sameer Goyal, Chandragouda Patil
Sickle cell disease is a frequent genetic anomaly characterized by altered molecular structure of hemoglobin resulting into crescent-like deformation of the red blood corpuscles. Neonatal jaundice is a frequent co-morbidity in sickle cell disease. Phototherapy induces isomerization of bilirubin rendering it extractable through urine and hence it is used as a routine treatment of neonatal jaundice. An exposure to light phototherapy as a treatment of neonatal jaundice induces oxidative stress. It is hypothesized that such exposure of neonates with sickle cell disease to the blue light phototherapy as a treatment of neonatal jaundice induces severe oxidative stress and increases the levels of proinflammatory cytokines...
November 2016: Medical Hypotheses
https://www.readbyqxmd.com/read/28079652/long-term-survival-and-causes-of-late-death-in-children-treated-with-extracorporeal-membrane-oxygenation
#6
Viktor von Bahr, Jan Hultman, Staffan Eksborg, Roxana Gerleman, Øyvind Enstad, Björn Frenckner, Håkan Kalzén
OBJECTIVE: Extracorporeal membrane oxygenation has been used in patients with severe circulatory or respiratory failure since the 1970s, but the knowledge on long-term survival in this group is scarce. The aim of the present study was to investigate the 10-year survival rates and causes of late death in children treated with extracorporeal membrane oxygenation. DESIGN: Single-center, retrospective cohort study. SETTING: Tertiary referral center for extracorporeal life support...
March 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/27289085/hemophagocytic-lymphohistiocytosis-pathogenesis-diagnosis-and-management
#7
REVIEW
Akira Morimoto, Yozo Nakazawa, Eiichi Ishii
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that is classified into primary and secondary HLH. Primary HLH consists of monogenic disorders that mainly affect the perforin-mediated cytotoxicity of cytotoxic T lymphocytes and natural killer cells. Secondary HLH occurs as a complication in various settings such as infection, malignancy, autoimmune disease, and post-allogeneic hematopoietic stem cell transplantation. Both primary and secondary HLH are characterized by uncontrolled hypercytokinemia that results in myelosuppression and vascular endothelium damage...
September 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/27571128/discontinuation-of-folic-acid-supplementation-in-young-patients-with-sickle-cell-anemia
#8
Giang-Kim T Nguyen, Angela Lewis, Carol Goldener, Brenda Reed, Robin Yates Dulman, Elizabeth Yang
Folic acid (FA) is commonly prescribed for patients with sickle cell anemia, but evidence for the efficacy of this practice is lacking. We stopped FA supplementation and measured red blood cell folate levels after discontinuation of FA in 72 patients with clinically severe forms of sickle cell disease. We compared hemoglobin and reticulocyte counts before and after FA discontinuation in 51 of those patients, the majority of whom were on hydroxyurea. No patients had red blood cell folate levels below normal and no significant difference in hemoglobin levels (P=0...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27647842/clinical-presentation-of-childhood-leukaemia-a-systematic-review-and-meta-analysis
#9
REVIEW
Rachel T Clarke, Ann Van den Bruel, Clare Bankhead, Christopher D Mitchell, Bob Phillips, Matthew J Thompson
OBJECTIVE: Leukaemia is the most common cancer of childhood, accounting for a third of cases. In order to assist clinicians in its early detection, we systematically reviewed all existing data on its clinical presentation and estimated the frequency of signs and symptoms presenting at or prior to diagnosis. DESIGN: We searched MEDLINE and EMBASE for all studies describing presenting features of leukaemia in children (0-18 years) without date or language restriction, and, when appropriate, meta-analysed data from the included studies...
October 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/26238275/novel-marker-for-the-detection-of-sickle-cell-nephropathy-soluble-fms-like-tyrosine-kinase-1-sflt-1
#10
Ilham Youssry, Samuel Makar, Rania Fawzy, Manal Wilson, Ghada AbdAllah, Eman Fathy, Happy Sawires
BACKGROUND: Given the burden and poor outcome of end-stage renal disease in sickle cell disease (SCD), early markers of sickle cell nephropathy (SN) are desirable. Disordered angiogenesis underlies many complications of SCD. We aimed to determine the relationship between serum FMS-like tyrosine kinase-1 (sFLT-1) and other biomarkers of renal damage for the early diagnosis of SN. METHODS: Forty-seven SCD patients and 49 healthy controls were enrolled. Microalbuminuria was determined in patient urine samples...
December 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27348695/critical-care-of-patients-with-cancer
#11
Alexander Shimabukuro-Vornhagen, Boris Böll, Matthias Kochanek, Éli Azoulay, Michael S von Bergwelt-Baildon
Answer questions and earn CME/CNE The increasing prevalence of patients living with cancer in conjunction with the rapid progress in cancer therapy will lead to a growing number of patients with cancer who will require intensive care treatment. Fortunately, the development of more effective oncologic therapies, advances in critical care, and improvements in patient selection have led to an increased survival of critically ill patients with cancer. As a consequence, critical care has become an important cornerstone in the continuum of modern cancer care...
June 27, 2016: CA: a Cancer Journal for Clinicians
https://www.readbyqxmd.com/read/26732078/anemia-and-transfusion-in-the-neonate
#12
REVIEW
Raffaella Colombatti, Laura Sainati, Daniele Trevisanuto
Neonatal anemia is a frequent occurrence in neonatal intensive care units. Red blood cell transfusion criteria in case of blood loss are clearly defined but optimal hemoglobin or hematocrit thresholds of transfusion for anemia due to decreased production or increased destruction are less evident. This review focuses on the causes of anemia in the newborn period and the most recent evidence-based treatment options, including transfusion and erythropoiesis-stimulating agents.
February 2016: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/27198469/regular-long-term-red-blood-cell-transfusions-for-managing-chronic-chest-complications-in-sickle-cell-disease
#13
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Ian R Hambleton, Gavin Cho
BACKGROUND: Sickle cell disease is a genetic haemoglobin disorder, which can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Sickle cell disease is one of the most common severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. The two most common chronic chest complications due to sickle cell disease are pulmonary hypertension and chronic sickle lung disease. These complications can lead to morbidity (such as reduced exercise tolerance) and increased mortality...
May 20, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27196265/early-discharge-of-neutropenic-pediatric-oncology-patients-admitted-with-fever
#14
Melanie A Villanueva, Keith J August
BACKGROUND: Fever and neutropenia (FN) is a common complication of pediatric oncology therapy and accounts for a large number of hospital admissions. Standard therapy for FN includes hospital admission and empiric antibiotics. Strict adherence to this practice leads to prolonged hospitalizations that may be unnecessary for patients at low risk of having an underlying significant infection. PROCEDURE: Children admitted with FN could be discharged after a minimum of 48 hr with no further antibiotic therapy once they had been afebrile for 24 hr with negative blood cultures from initial presentation, regardless of their neutrophil count...
October 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/26836905/chronic-pulmonary-complications-of-sickle-cell-disease
#15
REVIEW
Alem Mehari, Elizabeth S Klings
Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100,000 individuals, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes include HbSC, composed of one HbS allele and one HbC (glutamate-to-lysine mutation) allele; and HbS-β-thalassemia(0) or HbS-β-thalassemia(+), composed of one HbS allele and one β-thalassemia allele with absent or reduced β-chain production, respectively...
May 2016: Chest
https://www.readbyqxmd.com/read/23800659/red-cell-indices-differentiation-between-%C3%AE-thalassemia-trait-and-iron-deficiency-anemia-and-application-to-sickle-cell-disease-and-sickle-cell-thalassemia
#16
Chaima Abdelhafidh Sahli, Amina Bibi, Faida Ouali, Sondess Hadj Fredj, Boutheina Dakhlaoui, Rym Othmani, Naouel Laouini, Latifa Jouini, Fekria Ouenniche, Hajer Siala, Imed Touhami, Mariem Becher, Slaheddine Fattoum, Nour El Houda Toumi, Taieb Messaoud
BACKGROUND: In Tunisia, thalassemia and sickle cell disease represent the most prevalent monogenic hemoglobin disorders with 2.21% and 1.89% of carriers, respectively. This study aims to evaluate the diagnosis reliability of a series of red blood cell indices and parameters in differentiation of beta-thalassemia trait (β-TT) from iron deficiency anemia (IDA) and between homozygous sickle cell disease (SS) and sickle cell-thalassemia (ST). METHODS: The study covered 384 patients divided into three groups...
November 2013: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/23356250/influence-of-lime-juice-on-the-severity-of-sickle-cell-anemia
#17
RANDOMIZED CONTROLLED TRIAL
Samuel Ademola Adegoke, Umar Abdullahi Shehu, Lasisi Oluwafemi Mohammed, Yunusa Sanusi, Oyeku Akibu Oyelami
BACKGROUND AND OBJECTIVES: The pain in sickle cell anemia (SCA) is often triggered by dehydration, acidosis, and fever that are usually due to malaria. Intake of lime juice was recently demonstrated to facilitate clearance of the malaria parasite. It was therefore sought to determine whether regular intake of lime juice will ameliorate crisis, especially recurrent bone pain. DESIGN: In this preliminary, open-labeled, randomized study, the effects of lime juice on the clinical and some laboratory characteristics of children with SCA were tested...
June 2013: Journal of Alternative and Complementary Medicine: Research on Paradigm, Practice, and Policy
https://www.readbyqxmd.com/read/27127298/glucocorticoid-selection-for-pediatric-all
#18
Stephen P Hunger
No abstract text is available yet for this article.
April 28, 2016: Blood
https://www.readbyqxmd.com/read/27087396/daily-iron-supplementation-for-improving-anaemia-iron-status-and-health-in-menstruating-women
#19
REVIEW
Michael Sze Yuan Low, Joanna Speedy, Claire E Styles, Luz Maria De-Regil, Sant-Rayn Pasricha
BACKGROUND: Iron-deficiency anaemia is highly prevalent among non-pregnant women of reproductive age (menstruating women) worldwide, although the prevalence is highest in lower-income settings. Iron-deficiency anaemia has been associated with a range of adverse health outcomes, which restitution of iron stores using iron supplementation has been considered likely to resolve. Although there have been many trials reporting effects of iron in non-pregnant women, these trials have never been synthesised in a systematic review...
April 18, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/26819490/clinical-applications-of-hemolytic-markers-in-the-differential-diagnosis-and-management-of-hemolytic-anemia
#20
REVIEW
W Barcellini, B Fattizzo
Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an indicator of marrow compensatory response, elevated lactate dehydrogenase, a marker of intravascular hemolysis, reduced haptoglobin, and unconjugated hyperbilirubinemia. The direct antiglobulin test is the cornerstone of autoimmune forms, and blood smear examination is fundamental in the diagnosis of congenital membrane defects and thrombotic microangiopathies...
2015: Disease Markers
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