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hemolytic anemia treatment

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https://www.readbyqxmd.com/read/28918421/macrophage-activation-syndrome-at-the-onset-of-glucocorticoid-resistant-systemic-lupus-erythematosus-a-case-report
#1
Delia Tulbă, Marius Balea, Cristian Băicuș
INTRODUCTION: Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory state mediated by uncontrolled cytokine storm and haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids (GCs) are the cornerstone of SLE therapy. However, in some cases high doses of GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading to significant side effects. CASE REPORT: A 28-year-old Romani male was admitted to our hospital for polyarthralgia, polyserositis and fatigability...
September 16, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28905254/use-of-eculizumab-in-a-systemic-lupus-erythemathosus-patient-presenting-thrombotic-microangiopathy-and-heterozygous-deletion-in-cfhr1-cfhr3-a-case-report-and-systematic-review
#2
REVIEW
Maria Izabel de Holanda, Luis Cristóvão Pôrto, Teresa Wagner, Luis Fernando Christiani, Lilian M P Palma
The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports...
September 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28880867/oral-tetrahydrouridine-and-decitabine-for-non-cytotoxic-epigenetic-gene-regulation-in-sickle-cell-disease-a-randomized-phase-1-study
#3
RANDOMIZED CONTROLLED TRIAL
Robert Molokie, Donald Lavelle, Michel Gowhari, Michael Pacini, Lani Krauz, Johara Hassan, Vinzon Ibanez, Maria A Ruiz, Kwok Peng Ng, Philip Woost, Tomas Radivoyevitch, Daisy Pacelli, Sherry Fada, Matthew Rump, Matthew Hsieh, John F Tisdale, James Jacobberger, Mitch Phelps, James Douglas Engel, Santhosh Saraf, Lewis L Hsu, Victor Gordeuk, Joseph DeSimone, Yogen Saunthararajah
BACKGROUND: Sickle cell disease (SCD), a congenital hemolytic anemia that exacts terrible global morbidity and mortality, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs). Fetal hemoglobin (HbF) interferes with this polymerization, but HbF is epigenetically silenced from infancy onward by DNA methyltransferase 1 (DNMT1). METHODS AND FINDINGS: To pharmacologically re-induce HbF by DNMT1 inhibition, this first-in-human clinical trial (NCT01685515) combined 2 small molecules-decitabine to deplete DNMT1 and tetrahydrouridine (THU) to inhibit cytidine deaminase (CDA), the enzyme that otherwise rapidly deaminates/inactivates decitabine, severely limiting its half-life, tissue distribution, and oral bioavailability...
September 2017: PLoS Medicine
https://www.readbyqxmd.com/read/28878911/severe-autoimmune-hemolytic-anemia-during-pegylated-interferon-plus-ribavirin-treatment-for-chronic-hepatitis-c-a-case-report
#4
Shasha Wang, Eryun Qin, Yixiao Zhi, Rui Hua
We report a rare case of severe autoimmune hemolytic anemia triggered by pegylated interferon during combination therapy for chronic HCV. This case demonstrated that interferon can de novo induce autoimmune hemolytic anemia during therapy for chronic hepatitis C in a previously healthy patient.
September 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28878866/association-of-autoimmune-hepatitis-type-1-in-a-child-with-evans-syndrome
#5
Chaowapong Jarasvaraparn, Hamayun Imran, Abdul Siddiqui, Felicia Wilson, David A Gremse
Autoimmune hepatitis (AIH) is a progressive liver disease that is often associated with extrahepatic autoimmune disorders. Evans syndrome (ES) is a rare autoimmune disorder, which is characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Association of AIH with ES is rare, especially in children. We report a 3-year-old female with a past medical history of ES who presented with jaundice and significant transaminitis due to AIH type 1. She required multiple treatments with steroids as well as azathioprine, intravenous immunoglobulin and a course of rituximab...
August 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28872489/a-case-of-pure-red-cell-aplasia-during-nivolumab-therapy-for-cardiac-metastatic-melanoma
#6
Akihiko Yuki, Tatsuya Takenouchi, Sumiko Takatsuka, Takuro Ishiguro
Nivolumab is an antibody against programmed cell death 1 and functions as an immune checkpoint inhibitor for various malignancies, including unresectable melanomas. Nivolumab causes several immune-related adverse events, which typically include skin rash, pneumonitis, thyroid dysfunction, hepatitis, and colitis; in rare cases, anemia may be present. There are several reports of autoimmune hemolytic anemia that has developed in response to nivolumab; however, there are few reports of pure red cell aplasia (PRCA)...
September 1, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28871669/facilitated-subcutaneous-immunoglobulin-fscig-in-autoimmune-cytopenias-associated-with-common-variable-immunodeficiency
#7
Veronica Pedini, Isabella Savore, Giovanna Maria Danieli
BACKGROUND: Common variable immunodeficiency (CVID) is the most common symptomatic primary immune deficiency of adulthood. Besides recurrent infections, autoimmune disorders-mainly cytopenias-affect 30% of patients with CVID. OBJECTIVES: To describe the efficacy and safety of facilitated subcutaneous immunoglobulin (fSCIg), which is a combination of 10% [human] SCIg with recombinant human hyaluronidase for the treatment of CVID-linked cytopenias. METHODS: We describe four women (mean age 54 years) with CVID associated with idiopathic thrombocytopenic purpura (ITP) (n=3) and autoimmune hemolytic anemia (AIHA) (n=1)...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28838521/aortic-valve-replacement-as-a-trigger-of-atypical-hemolytic-uremic-syndrome
#8
Konstantinos Markakis, Timm H Westhoff, Nikolaos Pagonas
Mechanical hemolysis is a frequent but usually harmless complication of aortic valve replacement. The most common reason is valvular leakage. This report presents atypical hemolytic uremic syndrome (aHUS) as an alternative cause of mechanical hemolysis after this procedure. aHUS is a complement-mediated disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. It necessitates immediate specific treatment including plasma exchange or complement inhibition to avoid an adverse outcome...
September 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28810311/-retrospective-analysis-of-13-cases-of-nocardiosis
#9
H Q Yang, H Z Shi, Z H Tong
Objective: To evaluate the clinical features, chest radiological manifestations, microbiological examination and treatments of nocardial disease. Methods: A retrospective study was conducted to analyze the data of patients with nocardial infection admitted to Beijing Chaoyang Hospital from January 2010 to January 2016. Results: The 13 patients, 6 males and 7 females, aged (51±17) years. Twelve cases were diagnosed with pulmonary nocardiosis, and 1 with disseminated nocardial infection. Most of these patients had complications: autoimmune diseases in 3 (2 with autoimmune hemolytic anemia and 1 with systemic lupus erythematosus), and bronchiectasis in 6 patients...
August 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28797532/assessment-of-in-vitro-antifungal-efficacy-and-in-vivo-toxicity-of-amphotericin-b-loaded-plga-and-plga-peg-blend-nanoparticles
#10
T C Moraes Moreira Carraro, C Altmeyer, N Maissar Khalil, R Mara Mainardes
Amphotericin B (AmB) is widely applied in treatment of systemic fungal infections. However, the emergence of severe adverse effects, such as nephrotoxicity, hepatotoxicity and hemolytic anemia, can limit its clinical use. Poly(lactide-co-glycolide) (PLGA) or poly(lactide-co-glycolide)-poly(ethylene glycol) (PLGA-PEG) blend nanoparticles containing AmB were developed with the aim to decrease AmB toxicity and propose the oral route for AmB delivery. Nanoparticles were characterized by particle size, polydispersity index, Fourier transform infrared spectroscopy, differential scanning calorimetry and X-ray diffraction analyses...
August 7, 2017: Journal de Mycologie Médicale
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#11
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28780041/quinine-induced-thrombotic-microangiopathy-a-report-of%C3%A2-19%C3%A2-patients
#12
Evaren E Page, Dustin J Little, Sara K Vesely, James N George
BACKGROUND: Quinine can cause diverse and severe immune-mediated adverse reactions, including thrombotic microangiopathy (TMA). Our objective was to describe the presenting features and long-term outcomes of patients with quinine-induced TMA. STUDY DESIGN: A case series of 19 patients with quinine-induced TMA treated with plasma exchange. SETTING & PARTICIPANTS: Patients with quinine-induced TMA initially suspected of having thrombotic thrombocytopenic purpura (TTP) were identified among patients enrolled in the Oklahoma TTP-Hemolytic Uremic Syndrome Registry...
August 3, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28770911/targeting-%C3%AE-cys93-in-hemoglobin-s-with-an-antisickling-agent-possessing-dual-allosteric-and-antioxidant-effects
#13
Tigist Kassa, Michael Brad Strader, Akito Nakagawa, Warren M Zapol, Abdu I Alayash
Sickle cell disease (SCD) is an inherited blood disorder caused by a β globin gene mutation of hemoglobin (HbS). The polymerization of deoxyHbS and its subsequent aggregation (into long fibers) is the primary molecular event which leads to red blood cell (RBC) sickling and ultimately hemolytic anemia. We have recently suggested that HbS oxidative toxicity may also contribute to SCD pathophysiology due to its defective pseudoperoxidase activity. As a consequence, a persistently higher oxidized ferryl heme is formed which irreversibly oxidizes "hotspot" residues (particularly βCys93) causing protein unfolding and subsequent heme loss...
September 20, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28766838/transfusion-transmitted-babesiosis-leading-to-severe-hemolysis-in-two-patients-with-sickle-cell-anemia
#14
Kristine Karkoska, James Louie, Abena O Appiah-Kubi, Lawrence Wolfe, Lorry Rubin, Sujatha Rajan, Banu Aygun
The intracellular parasites Babesia microti and Babesia duncani can be transmitted by blood transfusion and cause severe life-threatening hemolytic anemia in high-risk patients, including those with sickle cell disease. The rarity of the diagnosis, as well as its similar clinical presentation to delayed hemolytic transfusion reaction, may lead to a delay in diagnosis, as well as inappropriate treatment with steroids or other immunosuppressive agents. The morbidity caused by this disease in especially vulnerable populations justifies the need for a universal blood-screening program in endemic areas...
August 2, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28760888/ag-348-enhances-pyruvate-kinase-activity-in-red-blood-cells-from-patients-with-pyruvate-kinase-deficiency
#15
Charles Kung, Jeff Hixon, Penelope A Kosinski, Giovanni Cianchetta, Gavin Histen, Yue Chen, Collin Hill, Stefan Gross, Yaguang Si, Kendall Johnson, Byron DeLaBarre, Zhiyong Luo, Zhiwei Gu, Gui Yao, Huachun Tang, Cheng Fang, Yingxia Xu, Xiaobing Lv, Scott Biller, Shin-San Michael Su, Hua Yang, Janeta Popovici-Muller, Francesco Salituro, Lee Silverman, Lenny Dang
Pyruvate kinase (PK) deficiency is a rare genetic disease that causes chronic hemolytic anemia. There are currently no targeted therapies for PK deficiency. Here, we describe the identification and characterization of AG-348, an allosteric activator of PK that is currently in clinical trials for the treatment of PK deficiency. We demonstrate that AG-348 can increase the activity of wild-type and mutant PK enzymes in biochemical assays and in patient red blood cells treated ex vivo. These data illustrate the potential for AG-348 to restore the glycolytic pathway activity in patients with PK deficiency and ultimately lead to clinical benefit...
September 14, 2017: Blood
https://www.readbyqxmd.com/read/28740963/extra-adrenal-myelolipoma-with-hemolytic-anemia
#16
Nidal İflazoğlu, Orhan Üreyen, Mahir Keleş
Myelolipomas are rare benign tumors often detected as adrenal masses. Extra-adrenal myelolipomas are encountered even more rarely. The rate of detection of these lesions is increasing with improved radiological techniques. Because of their localization and morphological similarities to well differentiated liposarcomas, extra-adrenal myelolipomas need to be differentiated from other aggressive neoplasms. Preoperative imaging and percutaneous biopsy are important tools in the diagnosis of these lesions. We report a very rare case of an extra-adrenal perirenal myelolipoma associated with hemolytic anemia...
2017: Turk J Surg
https://www.readbyqxmd.com/read/28737602/treatment-of-congenital-thrombotic-thrombocytopenia-purpura-a-new-paradigm
#17
Louis M Aledort, Tammuella C Singleton, Paula J Ulsh
Congenital thrombotic thrombocytopenia purpura (cTTP) is a very rare disorder worldwide. Standard treatment of recognized cases has been to administer fresh frozen plasma as the source of ADAMTS13, to replenish the absent ADAMTS13 enzyme. An alternative source, a plasma-derived factor VIII concentrate used for hemophilia A, and found to contain this enzyme, was reported to be effective in 1 patient in the United States. We now report details on a US cohort of 8 cTTP patients who have been successfully treated for varying periods with a marketed antihemophilic factor concentrate Koate-DVI...
July 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28734789/weathered-mc252-crude-oil-induced-anemia-and-abnormal-erythroid-morphology-in-double-crested-cormorants-phalacrocorax-auritus-with-light-microscopic-and-ultrastructural-description-of-heinz-bodies
#18
Kendal E Harr, Fred L Cunningham, Chris A Pritsos, Karen L Pritsos, Thivanka Muthumalage, Brian S Dorr, Katherine E Horak, Katie C Hanson-Dorr, Karen M Dean, Dave Cacela, Andrew K McFadden, Jane E Link, Katherine A Healy, Pete Tuttle, Steven J Bursian
Injury assessment of birds following the Deepwater Horizon (DWH) oil spill in 2010 was part of the Natural Resource Damage Assessment. One reported effect was hemolytic anemia with the presence of Heinz bodies (HB) in birds, however, the role of route and magnitude of exposure to oil is unknown. The purpose of the present study was to determine if double-crested cormorants (Phalacocorax auritis; DCCO) exposed orally and dermally to artificially weathered crude oil would develop hemolytic anemia including HB and reticulocytosis...
July 19, 2017: Ecotoxicology and Environmental Safety
https://www.readbyqxmd.com/read/28723780/the-relationship-between-itpa-rs1127354-polymorphisms-and-efficacy-of-antiviral-treatment-in-northeast-chinese-chc-patients
#19
MULTICENTER STUDY
Zhenhua Liu, Song Wang, Wenqian Qi, Xu Wang, Derong Sun, Hongguang Wang, Yonggui Zhang, Zhongxie Li, Liying Zhu, Ping Zhao, Honghua Guo, Changyu Zhou, Jiangbin Wang
This prospective study investigated the relationship between 2 inosine triphosphatase (ITPA) polymorphisms (rs7270101 and rs1127354) and the efficacy of ribavirin-based antiviral therapy in hepatitis C virus (HCV)-infected Chinese patients.A total of 906 patients diagnosed with chronic hepatitis C receiving pegylated interferon (PEG-IFN) plus ribavirin combination therapy between January 2011 and January 2014 from 5 hepatitis centers in Northeast China were enrolled. The patients were divided into genotype 1 and non-genotype 1 groups according to the genotype of infected HCV...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28721106/adverse-reactions-in-leprosy-patients-who-underwent-dapsone-multidrug-therapy-a-retrospective-study
#20
Sanjeev Guragain, Namrata Upadhayay, Bishwa Mohan Bhattarai
OBJECTIVE: To investigate the occurrence and clinical characteristics of dapsone-related adverse drug reactions (ADRs) among leprosy patients who underwent multidrug therapy (MDT) from 2010 to 2013 in the western region of Nepal. METHODS: A retrospective review was carried out in the rehabilitation center. Data were collected from the record files of the hospital. RESULTS: From 2010 to 2013, there were 18 patients reported to have dapsone ADRs, with an occurrence rate of 0...
2017: Clinical Pharmacology: Advances and Applications
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