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hemolytic anemia treatment

Minke A E Rab, Brigitte A van Oirschot, Jennifer Bos, Tesy H Merkx, Annet C W van Wesel, Osheiza Abdulmalik, Martin K Safo, A Birgitta Versluijs, Maite E Houwing, Marjon H Cnossen, Jurgen Riedl, Roger E G Schutgens, Gerard Pasterkamp, Marije Bartels, Eduard J van Beers, Richard van Wijk
In sickle cell disease (SCD), sickle hemoglobin (HbS) polymerizes upon deoxygenation, resulting in sickling of red blood cells (RBCs). These sickled RBCs have strongly reduced deformability, leading to vaso-occlusive crises and chronic hemolytic anemia. To date, there are no reliable laboratory parameters or assays capable of predicting disease severity or monitoring treatment effects. We here report on the oxygenscan: a newly developed method to measure RBC deformability (expressed as Elongation Index - EI) as a function of pO2 ...
February 19, 2019: American Journal of Hematology
Asmaa M Zahran, Khalid I Elsayh, Khaled Saad, Mostafa M Embaby, Mervat A M Youssef, Yasser F Abdel-Raheem, Shaban M Sror, Shereen M Galal, Helal F Hetta, Mohamed Diab Aboul-Khair, Mohamd A Alblihed, Amira Elhoufey
Sickle cell disease (SCD) is a genetically inherited hemolytic anemia increasingly appreciated as a chronic inflammatory condition and hypercoagulable state with high thrombotic risk. It is associated with disturbed immune phenotype and function and circulating microparticles (MPs) derived from multiple cell sources. This study was carried out to determine MPs profiles in patients with sickle cell anemia (either on hydroxyurea (HU) therapy or those with no disease-modifying therapy) and to compare these profiles with healthy children...
January 2019: Clinical and Applied Thrombosis/hemostasis
Ting-Xiao Li, Fu-Ting Sun, Bao-Jun Ji
OBJECTIVE: To investigate the correlation of IgG subclasses with blood cell parameters in the patients with autoimmune hemolytic anemia (AIHA). METHODS: Thirty-four patients with AIHA (except C3d types) of immune complex type (IgG+C3d) and single IgG type, including 26 cases of primary AIHA and 8 cases of secondary AIHA from December 2010 to August 2016 in our hospital were selected and enrolled in AIHA group; 30 healthy persons were selected and enrolled in healthy control group...
February 2019: Zhongguo Shi Yan Xue Ye Xue za Zhi
Qiang Pei, Hao-Rui Shen, Wei Zhang, Dao-Bin Zhou
OBJECTIVE: To investigate the clinical value of erythrocyte life span in the diagnosis and treatment of lymphoma patients with anemia. METHODS: The clinical data of 45 patients with lymphoma diagnosed by Department of Hematology in Beijing Union Hospital from January 2017 to June 2017 were collected. The life of erythrocytes was detected by alveolar gas CO test. RESULTS: The incidence of anemia in patients with malignant lymphoma was 46.7%, 40...
February 2019: Zhongguo Shi Yan Xue Ye Xue za Zhi
Mohammed A Al-Hijji, Abdallah El Sabbagh, Mayra E Guerrero, Charanjit S Rihal, Mackram F Eleid
BACKGROUND: Treatment of hemodynamically significant mitral annular calcification (MAC) using transcatheter approaches is in the early learning phase. The occurrence of paravalvular leak (PVL) following transcatheter mitral valve in MAC is common. AIMS: To report the initial experience and techniques of percutaneous PVL closure after transcatheter valve in MAC. METHODS: This series includes five consecutive patients who underwent percutaneous PVL closure following transcatheter balloon expandable SAPIEN S3 valve in MAC...
February 9, 2019: Catheterization and Cardiovascular Interventions
Carlos Quintanilla-Bordás, Emma Castro-Izaguirre, María Carcelén-Gadea, María Marín
BACKGROUND: Drug-induced hemolytic anemia is a rare and potentially fatal complication of drug treatment. Specific laboratory tests are crucial to confirm the diagnosis. CASE REPORT: A 38-year-old woman on treatment with dimethyl fumarate for multiple sclerosis presented with a 7-day history of weakness and fatigue. Laboratory tests revealed profound hemolytic anemia with hemoglobin levels of 4.7 g/dL (reference, 12.5-16.0), decreased haptoglobin, increased reticulocyte count, and increased indirect bilirubin...
January 31, 2019: Transfusion
Jui-Ming Liu, Cheng-Ping Yu, Heng-Chang Chuang, Chun-Te Wu, Ren-Jun Hsu
BACKGROUND: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases. METHODS: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases...
January 28, 2019: Prostate Cancer and Prostatic Diseases
Thomas Pincez, Bénédicte Neven, Hubert Ducou Le Pointe, Pascale Varlet, Helder Fernandes, Albane Gareton, Guy Leverger, Thierry Leblanc, Hervé Chambost, Gérard Michel, Marlène Pasquet, Frédéric Millot, Olivier Hermine, Alexis Mathian, Marie Hully, Hélène Zephir, Mohamed Hamidou, Jean-Marc Durand, Yves Perel, Judith Landman-Parker, Fréderic Rieux-Laucat, Nathalie Aladjidi
PURPOSE: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. METHODS: OBS'CEREVANCE is a French, nationwide prospective cohort that includes children with chronic ITP, AIHA, and ES...
January 22, 2019: Journal of Clinical Immunology
Hira Shaikh, Muhammad Salman Faisal, Prerna Mewawalla
Historically known to be a disease of sailors and soldiers in the seventeenth and eighteenth century, scurvy is a rare nutritional deficiency in the developed world, but it can still be seen among the alcoholics and the malnourished. We present a case of a 39-year-old alcoholic male who presented with progressive fatigue and diffuse purpuric rash with scattered ecchymosis for 2 months. Blood work was remarkable for hemoglobin of 9.1 g/dl, which further dropped to 7 g/dl over the next few days. He was then found to have hemolysis on lab work...
January 22, 2019: International Journal of Hematology
Jo Howard, Claire Jane Hemmaway, Paul Telfer, D Mark Layton, John Porter, Moji Awogbade, Timothy Mant, Daniel D Gretler, Kobina Dufu, Athiwat Hutchaleelaha, Mira Patel, Vincent Siu, Sandra Dixon, Noel Landsman, Margaret Tonda, Joshua Lehrer-Graiwer
New treatments directly targeting polymerization of sickle hemoglobin (HbS), the proximate event in the pathophysiology of sickle cell disease (SCD), are needed to address the severe morbidity and early mortality associated with the disease. Voxelotor (GBT440) is a first-in-class, oral therapy specifically developed to treat SCD by modulating the affinity of hemoglobin for oxygen, thus inhibiting HbS polymerization and the downstream adverse effects of hemolytic anemia and vaso-occlusion. GBT440-001 was a phase 1/2 randomized, double-blind, placebo-controlled, single and multiple ascending dose study of voxelotor in adult healthy volunteers and patients with SCD which was followed by a single-arm, open-label extension study...
January 17, 2019: Blood
Armelle deLaforcade, Lenore Bacek, Marie-Claude Blais, Robert Goggs, Alex Lynch, Elizabeth Rozanski
OBJECTIVES: Thrombosis is a well-recognized phenomenon in dogs and cats with a significant impact on morbidity and mortality. Despite growing awareness of thrombosis and increased use of antithrombotic therapy, there is little information in the veterinary literature to guide the use of anticoagulant and antiplatelet medications. The goal of Domain 1 was to explore the association between disease and thrombosis in a number of conditions identified as potential risk factors in the current veterinary literature, to provide the basis for prescribing recommendations...
January 2019: Journal of Veterinary Emergency and Critical Care
Yu Du, Yi Mou
The mortality rate of cupric sulfate is relatively high in contrast to that of other heavy metals. Cases of orally ingested cupric sulfate poisoning are very rare, with a reported half lethal dose of 10 g. Cupric sulfate poisoning leads to gastrointestinal corrosion, intravascular hemolysis, hemolytic anemia, methemoglobinemia and acute renal and hepatic impairment. Without proper and prompt treatment, multiple organ failure and death occur. Here, we present the first report that removal of the excessive intravascular copper ions by plasmapheresis was accompanied by complete recovery...
November 29, 2018: American Journal of the Medical Sciences
Guntur Darmawan, Laniyati Hamijoyo, Amaylia Oehadian, Ria Bandiara, Lisda Amalia
A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission...
October 2018: Acta Medica Indonesiana
L L Fu, J Ma, J Y Ma, R Zhang, H Gu, Z P Chen, R H Wu
Objective: To explore the clinical features and points of diagnosis and treatment for congenital thrombotic thrombocytopenic purpura (TTP) in children. Methods: The clinical manifestations, laboratory tests, genetic analysis and treatments of 5 children with congenital TTP hospitalized in Beijing Children's Hospital, Capital Medical University from February 2015 to July 2017 were analyzed retrospectively. Results: Among the 5 children with congenital TTP diagnosed by genetic monitoring and enzymology, there were 1 male and 4 females, 3 cases had suspicious positive family history, the age of onset was several hours after birth (range several hours after birth to 28 months)...
January 2, 2019: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Satoko Oka, Masaharu Nohgawa
Autoimmune diseases, including autoimmune hemolytic anemia and immune thrombocytopenic purpura, have been described in patients with non-Hodgkin lymphoma (NHL) after immunochemotherapy. However, the underlying pathogenesis remains unclear. We examined NHL patients with autoimmune cytopenia and all patients were treated with rituximab-containing therapy. The present results showed reversed imbalances in helper/suppressor T-cell populations, and an immune system imbalance may have contributed to immunological abnormalities...
January 10, 2019: Acta Haematologica
Michael Scordo, Meier Hsu, Ann A Jakubowski, Gunjan L Shah, Christina Cho, Molly A Maloy, Scott T Avecilla, Esperanza Papadopoulos, Boglarka Gyurkocza, Hugo Castro-Malaspina, Roni Tamari, Richard J O'Reilly, Miguel Angel-Perales, Sergio A Giralt, Brian C Shaffer
Immune mediated cytopenias (IC), such as immune thrombocytopenia (ITP) and immune hemolytic anemia (IHA), are among the adverse events after allogeneic hematopoietic cell transplantation (allo-HCT). Previous reports suggest that in-vivo T-cell depletion may increase the incidence of IC after allo-HCT. We evaluated whether a strategy that reduces functional donor T-cells via ex-vivo CD34+ -selection associates with the development of IC in a cohort of 408 patients who underwent allo-HCT for hematologic malignancy...
January 6, 2019: Biology of Blood and Marrow Transplantation
Marie Scully, Spero R Cataland, Flora Peyvandi, Paul Coppo, Paul Knöbl, Johanna A Kremer Hovinga, Ara Metjian, Javier de la Rubia, Katerina Pavenski, Filip Callewaert, Debjit Biswas, Hilde De Winter, Robert K Zeldin
BACKGROUND: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets...
January 9, 2019: New England Journal of Medicine
Kerry Morrone, William Beau Mitchell, Deepa Manwani
Sickle cell disease is an inherited hemoglobinopathy characterized by hemolytic anemia, frequent painful episodes, poor quality of life, end organ damage and a shortened lifespan. Although the seminal event is the polymerization of the abnormal hemoglobin, the downstream pathophysiology of vaso-occlusion results from heterotypic interactions between the altered, adhesive sickle cell RBCs, neutrophils, endothelium, and platelets. Ischemia reperfusion injury, hemolysis and oxidant damage all contribute to heightened inflammation and activation of the hemostatic system...
April 2018: Seminars in Hematology
Yonas Raru, Mahmoud Abouzid, Julia Parsons, Fuad Zeid
Rasburicase is a recombinant urate-oxidase enzyme and is a very important medication for tumor lysis syndrome. Methemoglobinemia and hemolysis are known side effects of rasburicase that result from oxidative stress caused by hydrogen peroxide, a byproduct generated during the breakdown of uric acid to allantoin. Patients with G6PD deficiency have a decreased tolerance to oxidative stress and are therefore at a greater risk of hemolysis and methemoglobinemia with rasburicase. Our patient is a 56-year-old Caucasian male with a recent diagnosis of grade 2-3a non-Hodgkin's lymphoma who presented to our emergency department with shortness of breath and dark discoloration of urine...
2019: Respiratory Medicine Case Reports
Victoria Mainardi, Karina Rando, Marcelo Valverde, Daniela Olivari, Jorge Castelli, Gabriela Rey, Solange Gerona
INTRODUCTION AND AIM: Wilson's disease (WD) is an uncommon cause of acute liver failure (ALF). Our aim was to describe clinical features, diagnostic findings, treatments, and outcomes of patients with ALF due to WD. MATERIAL AND METHODS: Retrospective medical record reviews of all patients with ALF due to WD in eight years in Uruguay. RESULTS: WD was the cause of six (15%) of thirty-nine ALF cases. All patients were females, with a mean age of 18 years...
December 13, 2018: Annals of Hepatology
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