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https://www.readbyqxmd.com/read/29043829/postpartum-acute-hemolytic-transfusion-reactions-associated-with-anti-lea-in-two-pregnancies-complicated-by-preeclampsia
#1
Marcia Marchese
Lewis blood group antibodies, which are mostly naturally occurring and considered clinically insignificant, have rarely been documented as a cause of acute hemolytic transfusion reactions (AHTRs). This report presents two cases of AHTRs caused by anti-Lea occurring in postpartum black females (one group B, one group AB) whose pregnancies were complicated by preeclampsia. Neither anti-Lea was detected by automated solid-phase red cell adherence technology in pre-transfusion testing. Therefore, red blood cell units, compatible by electronic crossmatch, were issued and transfused...
September 2017: Immunohematology
https://www.readbyqxmd.com/read/29043828/a-lu-16-individual-with-antibodies
#2
Carole Éthier, Cynthia Parent, Anne-Sophie Lemay, Nadia Baillargeon, Geneviève Laflamme, Josée Lavoie, Josée Perreault, Maryse St-Louis
Antibodies against Lutheran blood group antigens have been observed during first-time pregnancy. Samples from a woman of African descent were tested in our immunohematology laboratory on several occasions since 2001. Her samples were phenotyped as Lu(a+b-), and anti-Lub was suspected but not identified. She was asked to make autologous donations in preparation for her delivery, which she did. In 2010, two antibodies were identified: anti-Lea and -Lub. Six years later, a third investigation was requested. This time, an antibody directed at a high-prevalence Lutheran antigen was found in addition to the anti-Lea and -Lub previously observed...
September 2017: Immunohematology
https://www.readbyqxmd.com/read/29042465/thrombotic-microangiopathy-and-the-kidney
#3
Vicky Brocklebank, Katrina M Wood, David Kavanagh
Thrombotic microangiopathy can manifest in a diverse range of diseases and is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ injury, including AKI. It can be associated with significant morbidity and mortality, but a systematic approach to investigation and prompt initiation of supportive management and, in some cases, effective specific treatment can result in good outcomes. This review considers the classification, pathology, epidemiology, characteristics, and pathogenesis of the thrombotic microangiopathies, and outlines a pragmatic approach to diagnosis and management...
October 17, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29040872/highly-elevated-plasma-level-of-von-willebrand-factor-accelerates-the-formation-of-platelet-thrombus-under-high-shear-stress-in-plasma-with-deficient-adamts13-activity
#4
Hideo Yagi, Naoko Yamaguchi, Yasuaki Shida, Mitsuhiko Sugimoto, Kazuo Tubaki, Yoshihiro Fujimura, Masanori Matsumoto
Upshaw-Schulman syndrome (USS) is a thrombo-hemorrhagic disease caused by congenital deficiency of ADAMTS13 due to ADAMTS13 gene mutations. USS is characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond dramatically to infusions of fresh frozen plasma. There are two phenotypic expressions of USS: one is the early-onset type and the other, the late-onset type, is asymptomatic during childhood with the first bout of thrombotic thrombocytopenic purpura (TTP) developing after adolescence or during adulthood...
October 10, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29040411/handoc-a-handy-score-to-determine-the-need-for-echocardiography-in-non-beta-hemolytic-streptococcal-bacteremia
#5
Torgny Sunnerhagen, Amanda Törnell, Maria Vikbrant, Bo Nilson, Magnus Rasmussen
Background: Non-beta-hemolytic streptococci (NBHS) are a common cause of infective endocarditis (IE). Echocardiography is used to diagnose IE, but it is not known which patients with NBHS bacteremia should undergo echocardiography. Method: Medical records of patients with NBHS bacteremia in southern Sweden from 2012-2014 were studied retrospectively. The patients were divided into two cohorts. In the first, correlations between the reported data and IE were studied...
October 10, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29032940/molecular-basis-of-%C3%AE-thalassemia
#6
REVIEW
Samaneh Farashi, Cornelis L Harteveld
α-Thalassemia is an inherited, autosomal recessive, disorder characterized by a microcytic hypochromic anemia. It is one of the most common monogenic gene disorders in the world population. The clinical severity varies from almost asymptomatic, to mild microcytic hypochromic, and to a lethal hemolytic condition, called Hb Bart's Hydrops Foetalis Syndrome. The molecular basis are usually deletions and less frequently, point mutations affecting the expression of one or more of the duplicated α-genes. The clinical variation and increase in disease severity is directly related to the decreased expression of one, two, three or four copies of the α-globin genes...
September 21, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/29019451/autoimmunity-in-a-cohort-of-471-patients-with-primary-antibody-deficiencies
#7
Gholamreza Azizi, Marzieh Tavakol, Hosein Rafiemanesh, Fatemeh Kiaee, Reza Yazdani, Amin Heydari, Kosar Abouhamzeh, Pardis Anvari, Sara Mohammadikhajehdehi, Laleh Sharifia, Yasser Bagheri, Hamed Mohammadi, Hassan Abolhassani, Asghar Aghamohammadi
OBJECTIVES: The aim of this study was to evaluate the frequency of autoimmunity in primary antibody deficiency (PAD). METHODS: A total of 471 patients with PADs enrolled in this retrospective cohort study. For all patients' demographic information, clinical records and laboratory data were collected to investigate autoimmune complications. RESULTS: Autoimmune disorders as the first presentation of immunodeficiency were recorded in 11 patients (2...
November 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28993505/factor-h-competitor-generated-by-gene-conversion-events-associates-with-atypical-hemolytic-uremic-syndrome
#8
Elena Goicoechea de Jorge, Agustín Tortajada, Sheila Pinto García, Sara Gastoldi, Héctor Martín Merinero, Jesús García-Fernández, Emilia Arjona, Mercedes Cao, Giuseppe Remuzzi, Marina Noris, Santiago Rodríguez de Córdoba
Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy caused by complement pathogenic variants, mainly affects the kidney microvasculature. A retrospective genetic analysis in our aHUS cohort (n=513) using multiple ligation probe amplification uncovered nine unrelated patients carrying a genetic abnormality in the complement factor H related 1 gene (CFHR1) that originates by recurrent gene conversion events between the CFH and CFHR1 genes. The novel CFHR1 mutants encode an FHR-1 protein with two amino acid substitutions, L290S and A296V, converting the FHR-1 C terminus into that of factor H (FH)...
October 9, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28991131/recurrent-atypical-hemolytic-uremic-syndrome-in-children-with-acute-lymphoblastic-leukemia-undergoing-maintenance-chemotherapy
#9
Geoffrey Cheng, Bulent Ozgonenel, Kanta Bhambhani, Gaurav Kapur, Richard J Smith, Süreyya Savaşan
Chemotherapy-associated myelosuppression and renal dysfunction is not uncommon during childhood acute lymphoblastic leukemia (ALL) therapy. Here we report 2 cases of atypical hemolytic uremic syndrome (aHUS) presenting with pancytopenia and renal dysfunction that developed during maintenance chemotherapy characterized by hypocomplementemia. Both cases experienced recurrence after resolution of the initial aHUS episode upon resumption of chemotherapy, raising a possible contributory role for chemotherapy in the disease pathogenesis...
October 4, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28986178/neuropharmacological-and-acute-toxicological-evaluation-of-ethanolic-extract-of-allamanda-cathartica-l-flowers-and-plumieride
#10
Tiago J Bonomini, Iandra Holzmann, Liliani Carolini Thiesen, Eduarda Fratoni, Ana Flavia Fischer Muller, Ruth M Lucinda-Silva, Rosendo Augusto Yunes, Angela Malheiros, Ana Elisa Gonçalves, Ana Paula Dalmagro, Kathryn Ana Bortolini Simão da Silva, Márcia Maria de Souza, José Roberto Santin
Psychiatric diseases affect more than 350 million people all over the world, and medicinal plants have been considered the basis for pharmacological research. The study investigates the anticonvulsant and antidepressant-like activities and acute toxicological effects of ethanolic extract of Allamanda cathartica flowers, and plumieride. The extract was analyzed by HPLC and plumieride was isolated. Toxicity studies were carried out on females Wistar rats (2000 mg/kg). Toxicity was evaluated by measuring biochemical parameters and conducting histopathological analysis...
October 4, 2017: Regulatory Toxicology and Pharmacology: RTP
https://www.readbyqxmd.com/read/28983018/phase-1-study-of-the-anti-cd22-immunotoxin-moxetumomab-pasudotox-for-childhood-acute-lymphoblastic-leukemia
#11
MULTICENTER STUDY
Alan S Wayne, Nirali N Shah, Deepa Bhojwani, Lewis B Silverman, James A Whitlock, Maryalice Stetler-Stevenson, Weili Sun, Meina Liang, Jie Yang, Robert J Kreitman, Mark C Lanasa, Ira Pastan
Novel therapies are needed to overcome chemotherapy resistance for children with relapsed/refractory acute lymphoblastic leukemia (ALL). Moxetumomab pasudotox is a recombinant anti-CD22 immunotoxin. A multicenter phase 1 study was conducted to determine the maximum-tolerated cumulative dose (MTCD) and evaluate safety, activity, pharmacokinetics, and immunogenicity of moxetumomab pasudotox in children, adolescents, and young adults with ALL (N = 55). Moxetumomab pasudotox was administered as a 30-minute IV infusion at doses of 5 to 50 µg/kg every other day for 6 (cohorts A and B) or 10 (cohort C) doses in 21-day cycles...
October 5, 2017: Blood
https://www.readbyqxmd.com/read/28982343/study-of-glucose-6-phosphate-dehydrogenase-deficiency-5-years-retrospective-egyptian-study
#12
Adel Abd Elhaleim Hagag, Ibrahim M Badraia, Mohamed S Elfarargy, Mohamed M Abd Elmageed, Ehab A Abo-Ali
BACKGROUND: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency worldwide that causes a spectrum of diseases including neonatal hyperbilirubinemia, acute and chronic hemolysis after exposure to an oxidative stress. AIM OF THE WORK: This five years retrospective study was carried out to study the demographic, clinical and laboratory data of 1000 patients with G6PD deficiency anemia registered in Hematology Unit, Pediatric Department, Tanta University Hospital...
October 3, 2017: Endocrine, Metabolic & Immune Disorders Drug Targets
https://www.readbyqxmd.com/read/28982104/repair-of-an-autologous-saphenous-vein-graft-aneurysm-ten-years-after-renal-artery-reconstruction-during-live-donor-renal-transplantation
#13
Markus Oelschlaeger, Ioannis Sokolakis, Charis Kalogirou, Lea Frey, Hubertus Riedmiller, Hubert Kübler, Richard Kellersmann, Daniel Vergho
Saphenous vein graft (SVG) aneurysms (SVGA) after renal transplantation represents a rare vascular complication with subsequent challenging multidisciplinary treatment. We present a case of a 30-year-old female who received a live donor kidney transplantation for end-stage renal disease that was caused due to the hemolytic uremic syndrome. Postoperatively, an insufficient graft perfusion due to an arterial kinking was noted and repaired using an autologous SVG interposition. Ten years later, a 3-cm aneurysm of the SVG at the anastomotic site with the common iliac artery was discovered...
October 6, 2017: Urologia Internationalis
https://www.readbyqxmd.com/read/28978852/new-developments-in-treatment-modalities-of-thrombotic-thrombocytopenic-purpura
#14
Masanori Matsumoto
Although thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, appropriate diagnosis and treatment result in the higher survival rate of >80%. TTP is usually suspected with thrombocytopenia and hemolytic anemia and is confirmed by a reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) <10%. TTP is classified as acquired if a patient tests positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are identified...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28975130/multiple-myeloma-as-the-underlying-cause-of-thrombotic-microangiopathy-leading-to-acute-kidney-injury-revisiting-a-very-rare-entity
#15
Savneek Chugh, Asim Kichloo, Firas Jafri, Liga Yusvirazi, Robert Lerner
Thrombotic microangiopathy (TMA) describes a pathological process of microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia, leading to end-organ ischemia and infarction, affecting particularly the kidney and brain. TMA is a pathological feature of a number of clinical disorders including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and atypical hemolytic uremic syndrome. Rare but important, TMA may also occur in malignancy, connective tissue disease, malignant hypertension, and renal transplantation (rejection or drug toxicity)...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28972879/challenging-clinical-presentations-of-pernicious-anemia
#16
Thein Hlaing Oo, Cristhiam Mauricio Rojas-Hernandez
Pernicious anemia (PA) is an autoimmune disease of multifactorial etiologies characterized by autoimmune chronic atrophic gastritis, cobalamin deficiency (CD) due to defective absorption of dietary cobalamin from the terminal ileum, and by the presence of intrinsic factor and parietal cell antibodies. PA is a very common cause of CD-related anemia worldwide. Despite advances in the understanding molecular biology and pathophysiology of PA, the diagnosis of PA remains challenging in many circumstances for many clinicians because of its diverse clinical manifestations and the limitations of currently available diagnostic tools...
September 2017: Discovery Medicine
https://www.readbyqxmd.com/read/28971506/hereditary-stomatocytosis-an-underdiagnosed-condition
#17
REVIEW
Immacolata Andolfo, Roberta Russo, Antonella Gambale, Achille Iolascon
Hereditary stomatocytoses are a wide class of hemolytic anemias characterized by alterations of ionic flux with increased cation permeability that results in inappropriate shrinkage or swelling of the erythrocytes, and water lost or gained osmotically. The last few years have been crucial for new acquisitions in this field in terms of identifying new causative genes and of studying their pathogenetic mechanisms. This review summarizes the main features of erythrocyte membrane transport diseases, dividing them into forms with either isolated erythroid phenotype (non-syndromic) or extra-hematological manifestations (syndromic), and focusing particularly on the most recent advances regarding dehydrated forms of hereditary stomatocytosis and familial pseudohyperkalemia...
October 3, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28970221/characterization-of-sorbitol-fermenting-enterohemorrhagic-escherichia-coli-o157-h-from-czech-patients-identifies-novel-plasmid-composition-not-previously-seen-in-german-isolates
#18
Andreas Bauwens, Monika Marejková, Barbara Middendorf-Bauchart, Rita Prager, Annelene Kossow, Wenlan Zhang, Helge Karch, Alexander Mellmann, Martina Bielaszewska
Sorbitol-fermenting (SF) enterohemorrhagic Escherichia coli (EHEC) O157:H(-) strains, first identified in Germany, have emerged as important pathogens throughout Europe. Besides chromosomally encoded Shiga toxin 2a, the major virulence factor, several putative virulence loci including the hly, etp, and sfp operons encoding EHEC hemolysin, type II secretion system, and Sfp fimbriae, respectively, are located on a 121 kb plasmid pSFO157 in German strains. Here, we report novel SF EHEC O157:H(-) strains isolated from patients in the Czech Republic...
September 29, 2017: Applied and Environmental Microbiology
https://www.readbyqxmd.com/read/28966502/rare-presentation-of-mixed-autoimmune-hemolytic-anemia-in-children-report-of-2-cases
#19
Preeti Rai, Geetika Sharma, Deeksha Singh, Jyoti Garg
Immune hemolytic anemia is characterized by clinical and laboratory features of hemolytic anemia with direct antiglobulin test (DAT) positivity. It could be autoimmune hemolytic anemia (AIHA), alloimmune, or drug-induced hemolysis based on the antigenic stimulus. Furthermore, based on thermal amplitude of autoantibody, AIHA is classified as warm (65%), cold (30%), and mixed (5%) type. Mixed AIHA is extremely rare in children and must be differentiated from warm AIHA with clinically insignificant cold agglutinins and cold hemagglutinin disease as their treatment is different...
October 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28958970/hemolytic-anemia-iron-deficiency-and-personal-history-of-deep-vein-thrombosis-consider-paroxysmal-nocturnal-hemoglobinuria
#20
Nicolas Gendron, Jean-Benoit Arlet, Pascale Gaussem, Isabelle Radford-Weiss, Sidonie Dupeux, Jérémie Rosain, Régis Peffault de La Tour, Luc Darnige
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder that must be correctly diagnose because it is a chronic disease with a real impact on the quality of life and the survival of the patients. PNH screening of all patients with anemia or thrombosis is not recommended. We report the case of a 71-year-old male patient referred for chronic anemia. Anemia work-up revealed a misunderstood association of a hemolytic anemia with a negative direct antiglobulin test and iron deficiency...
October 1, 2017: Annales de Biologie Clinique
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