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Autoimmune Anaemia

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https://read.qxmd.com/read/30784322/cd5-b-lymphocytes-secrete-il-10-rather-than-tgf-%C3%AE-1-which-control-the-immune-response-in-autoimmune-haemolytic-anaemia-evans-syndrome
#1
Manjun Zhao, Ningning Duan, Yi Wang, Hongli Zhu, Hong Liu, Huaquan Wang, Limin Xing, Zonghong Shao
OBJECTIVE: To investigate the quantity and secretion function of cytokines-secreted CD5+ B lymphocytes in Autoimmune Haemolytic Anaemia (AIHA)/Evans syndrome (ES) patients. METHODS: Twenty-five untreated AIHA/ES patients, 28 remission AIHA/ES patients and 25 healthy controls (HCs) were enrolled in this study. The quantity of CD5+ B lymphocytes which produce interleukin-10 (IL-10) (CD5+ IL-10+ ) and transforming growth factor (TGF-β1) (CD5+ TGF-β1+ ) were detected by flow cytometry (FCM)...
February 20, 2019: Autoimmunity
https://read.qxmd.com/read/30779476/hla-dq2-homozygosis-increases-ttga-levels-at-diagnosis-but-does-not-influence-the-clinical-phenotype-of-coeliac-disease-a-multicentre-study
#2
Judit Bajor, Zsolt Szakács, Márk Juhász, Mária Papp, Dorottya Kocsis, Éva Szegedi, Ildikó Földi, Nelli Farkas, Péter Hegyi, Áron Vincze
BACKGROUND AND PURPOSE: Magnitude of gluten-specific T-cell responses in coeliac disease (CD) might be dependent on HLA-DQ2 gene dose. We aimed to investigate the effects of HLA-DQB1*02 allele dose on clinical outcomes. METHODS: We reviewed the charts of all coeliac patients attending to three Hungarian university clinics after 1997 and included those patients, who (a) were diagnosed with CD, (b) underwent high-resolution HLA typing and (c) were ≥18 years at the time of data collection...
February 19, 2019: International Journal of Immunogenetics
https://read.qxmd.com/read/30659005/rare-presentation-of-iron-deficiency-anaemia-with-cerebral-venous-sinus-thrombosis-in-a-middle-aged-woman
#3
Amna Bibi, Chathuri Liyanapthirana, Sajjad Khan
This is a case of a female patient presented with a mild headache associated with recurrent episodes of nausea and vomiting, confusion and right-sided hemiparesis, diagnosed of haemorrhagic venous infarction from the thrombosis of left transverse and sigmoid sinus and probably the left vein of Labbe. She had severe microcytic hypochromic anaemia due to iron deficiency, without any other risk factors for cerebral venous thrombosis such as trauma history, infections, coagulation disorders or autoimmune diseases...
January 17, 2019: BMJ Case Reports
https://read.qxmd.com/read/30630540/identifying-and-improving-adherence-to-the-gluten-free-diet-in-people-with-coeliac-disease
#4
H Muhammad, S Reeves, Y M Jeanes
Coeliac disease (CD) is an autoimmune gastrointestinal disorder whereby the ingestion of gluten, a storage protein found in wheat, barley and rye, causes damage to intestinal mucosa with resultant malabsorption, increased risk of anaemia and osteoporosis. Worldwide estimates suggest 1% of the population have CD. With no cure, the only treatment is a gluten-free diet (GFD). Adhering to a GFD can be very challenging; it requires knowledge, motivation and modified behaviours. Assessing adherence to a GFD is methodologically challenging...
January 11, 2019: Proceedings of the Nutrition Society
https://read.qxmd.com/read/30567242/metastatic-lung-adenocarcinoma-associated-thrombotic-microangiopathy-in-a-renal-transplant-recipient
#5
Eswari Vilayur, Jillian de Malmanche, Paul Trevillian, David Ferreira
Thrombotic microangiopathy (TMA) after renal transplantation can be a diagnostic challenge. TMA can occur with calcineurin inhibitors, allograft rejection, infection, mutations in complement regulatory proteins and autoimmunity. A 52-year-old male renal transplant recipient presented with extensive deep vein thrombosis. He developed transfusion-dependent microangiopathic haemolytic anaemia with thrombocytopenia. He did not respond calcineurin inhibitor cessation, eculizumab or plasma exchange. ADAMTS13 and complement levels were normal...
December 13, 2018: BMJ Case Reports
https://read.qxmd.com/read/30567234/severe-refractory-idiopathic-warm-autoimmune-haemolytic-anaemia-responsive-to-complement-inhibition-with-eculizumab
#6
Lucy Neave, Andrew J Wilson, Maxine Lissack, Marie Scully
We report a case of severe idiopathic warm autoimmune haemolytic anaemia (wAIHA) which was initially poorly responsive to treatment with corticosteroids, intravenous immunoglobulin, mycophenolate mofetil and rituximab, and required transfusion of more than 30 units of red cells over 12 weeks. Off-label use of the terminal complement pathway inhibitor, eculizumab, led to rapid amelioration of the haemolysis, presumably by the inhibition of an intravascular component, and allowed time for slower acting immunosuppressive agent to take effect...
December 13, 2018: BMJ Case Reports
https://read.qxmd.com/read/30567221/rifampicin-not-always-an-innocent-drug
#7
Dafni Sveroni, Aggelos Stefos, Eirini I Rigopoulou, Georgios N Dalekos
Rifampicin has been widely used due to its broad antibacterial spectrum. Acute haemolysis is a rarely encountered complication of rifampicin. A 58-year-old woman was admitted to our department because of high-grade fever with rigors, accompanied by abdominal and lumbar pain and laboratory evidence of acute haemolysis. She had been treated for brucellosis initially with doxycycline and streptomycin. Due to subsequent appearance of myositis, ciprofloxacin and rifampicin were added for treatment of localised brucellosis...
December 9, 2018: BMJ Case Reports
https://read.qxmd.com/read/30564730/diagnostics-in-anaemia-of-chronic-disease-in-general-practice-a-real-world-retrospective-cohort-study
#8
Annemarie Schop, Karlijn Stouten, Ron van Houten, Jürgen Riedl, Joost van Rosmalen, Patrick Je Bindels, Mark-David Levin
Background: Limited research has been performed that focused on the diagnosis of the underlying cause of anaemia of chronic disease (ACD) in general practice or on prevalence data of the underlying causes of ACD in general practice, although this is one of the most common types of anaemia. Aim: To clarify the diagnostic strategies of GPs in patients newly diagnosed with ACD and to determine the most common underlying causes. Design & setting: Retrospective cohort study...
October 2018: BJGP Open
https://read.qxmd.com/read/30528137/haematological-immune-related-adverse-events-induced-by-anti-pd-1-or-anti-pd-l1-immunotherapy-a-descriptive-observational-study
#9
Nicolas Delanoy, Jean-Marie Michot, Thibault Comont, Nora Kramkimel, Julien Lazarovici, Romain Dupont, Stéphane Champiat, Claude Chahine, Caroline Robert, Charles Herbaux, Benjamin Besse, Aude Guillemin, Christine Mateus, Patricia Pautier, Philippe Saïag, Emanuela Madonna, Marie Maerevoet, Jean-Christophe Bout, Charlotte Leduc, Pascal Biscay, Gilles Quere, Charlée Nardin, Mikael Ebbo, Laurence Albigès, Grégoire Marret, Virginie Levrat, Cécile Dujon, Jacques Vargaftig, Salim Laghouati, Laure Croisille, Anne-Laure Voisin, Bertrand Godeau, Christophe Massard, Vincent Ribrag, Aurélien Marabelle, Marc Michel, Olivier Lambotte
BACKGROUND: Anti-programmed cell death 1 (PD-1) and anti-programmed cell death ligand 1 (PD-L1) antibodies are novel immunotherapies for cancer that can induce immune-related adverse events (irAEs). These adverse events can involve all organs, including the haemopoietic system. Thus far, haematological irAEs (haem-irAEs) have not been extensively characterised. This study aims to provide a comprehensive report of the haem-irAEs induced by anti-PD-1 or anti-PD-L1. METHODS: In this descriptive observational study, we included consecutive patients aged at least 18 years with grade 2 or worse haem-irAEs induced by anti-PD-1 or anti-PD-L1 immunotherapy registered in three French pharmacovigilance databases: the Registre des Effets Indésirables Sévères des Anticorps Monoclonaux Immunomodulateurs en Cancérologie (REISAMIC; a prospective registry of patients treated with anti-PD-1 or anti-PD-L1 at a single centre), the ImmunoTOX committee of Gustave Roussy (a national referral database of suspected irAEs in patients treated with immunotherapy), and the registry of the Centre de Référence des Cytopénies Auto-Immunes de l'Adulte (CeReCAI; a national database of autoimmune cytopenias)...
December 4, 2018: Lancet Haematology
https://read.qxmd.com/read/30479781/two-male-siblings-with-a-novel-lrba-mutation-presenting-with-different-findings-of-ipex-syndrome
#10
Sanem Eren Akarcan, Neslihan Edeer Karaca, Guzide Aksu, Ayca Aykut, Deniz Yilmaz Karapinar, Funda Cetin, Yesim Aydinok, Elif Azarsiz, Eleonora Gambineri, Ozgur Cogulu, Ezgi Ulusoy Severcan, Hudaver Alper, Necil Kutukculer
Introduction: LPS-responsive beige-like anchor (LRBA) protein deficiency is a disease of immune dysregulation with autoimmunity affecting various systems. Case Presentation: Two male siblings with a novel LRBA mutation had different primary findings at admission: the younger sibling had chronic early-onset diarrhoea and the elder one had autoimmune haemolytic anaemia. During long-term follow-up for IPEX phenotype, both developed hypogammaglobulinaemia, enteropathy and lung involvement...
October 2018: JMM Case Reports
https://read.qxmd.com/read/30477053/thiopurine-s-methyltransferase-tpmt-mutation-prevalence-and-myelosuppression-frequency-in-north-indian-patients-with-autoimmune-disorders
#11
Garg Ambar, Arya Vandana, Duggal Lalit, Kotwal Jyoti
Background: For many years, azathioprine and its active metabolite 6-merceptopurine are used as immunosuppressants for treatment of autoimmune disorders. However, azathioprine has low therapeutic index with myelosuppression as its predominant toxicity which is linked with thiopurine S-methyltransferase (TPMT) enzyme activity, which is involved in drug metabolism. TPMT activity is controlled by variants in TPMT gene. We aimed to estimate prevalence of TPMT gene mutations in North Indian patients with autoimmune disorders and to assess myelosuppression in these patients...
May 2018: Journal of the Association of Physicians of India
https://read.qxmd.com/read/30467085/fetal-stroke-and-cerebrovascular-disease-advances-in-understanding-from-lenticulostriate-and-venous-imaging-alloimmune-thrombocytopaenia-and-monochorionic-twins
#12
REVIEW
Fenella J Kirkham, Dimitrios Zafeiriou, David Howe, Philippa Czarpran, Ashley Harris, Roxanna Gunny, Brigitte Vollmer
Fetal stroke is an important cause of cerebral palsy but is difficult to diagnose unless imaging is undertaken in pregnancies at risk because of known maternal or fetal disorders. Fetal ultrasound or magnetic resonance imaging may show haemorrhage or ischaemic lesions including multicystic encephalomalacia and focal porencephaly. Serial imaging has shown the development of malformations including schizencephaly and polymicrogyra after ischaemic and haemorrhagic stroke. Recognised causes of haemorrhagic fetal stroke include alloimmune and autoimmune thrombocytopaenia, maternal and fetal clotting disorders and trauma but these are relatively rare...
September 11, 2018: European Journal of Paediatric Neurology: EJPN
https://read.qxmd.com/read/30370811/a-rare-case-of-coombs-negative-autoimmune-haemolytic-anaemia-in-pregnancy
#13
Kazuki Takasaki, Morikazu Miyamoto, Masashi Yoshida, Hiroaki Soyama, Masashi Takano, Kenichi Furuya
No abstract text is available yet for this article.
October 27, 2018: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://read.qxmd.com/read/30317320/rubrivigilance-in-diabetes
#14
Sanjay Kalra, Sundeep Ruder
Anaemia and diabetes have a multifaceted relationship. Their co-existence contributes to each other's etiopathogenesis, natural history, clinical presentation and prognosis. Anaemia may occur in autoimmune disease that coexists with type 1 diabetes, in erythropoietin deficient and in erythropoietin hyporesponsive states. Iron deficiency, haemolytic and megaloblastic anaemia occur in diabetes through various mechanisms. Anaemia is associated with, and contributes to, worsening of both microvascular and macrovascular complications...
July 2018: JPMA. the Journal of the Pakistan Medical Association
https://read.qxmd.com/read/30293518/autoimmune-haemolytic-anaemia-associated-with-p-vivax-malaria
#15
Sunil Taneja, Neha Agarwal
Autoimmune haemolytic anaemia due to malaria or following its treatment with artesunate is rare. A child presented with severe anaemia after being treated with artesunate for P. vivax malaria. Blood transfusion was difficult as cross-matching showed major incompatibility; group O negative blood under the cover of steroids was transfused. Oral steroids were given for six weeks. The patient made a complete recovery.
October 6, 2018: Tropical Doctor
https://read.qxmd.com/read/30237633/thrombotic-thrombocytopenic-purpura-as-the-first-presentation-in-systemic-lupus-erythematosus
#16
Opeyemi Folorunsho Bamidele, Richard Oluyinka Akintayo, Mathew Olumuyiwa Bojuwoye, Tolulope Oyetunde Alabi, Foluke Comfort Akintayo, Oluwakemi Victoria Bamidele
Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease characterized by severe thrombocytopenia, microangiopathic haemolytic anaemia, neurologic abnormalities, renal insufficiency, and fever commonly associated with infections, malignancy, drugs, and autoimmune diseases. Coexistence of TTP with systemic lupus erythematosus (SLE) is extremely rare, and the emergence of the full manifestation of SLE starting with TTP is even rarer. Black people due to genetic risk factors are at increased risk of TTP...
2018: Reumatologia
https://read.qxmd.com/read/30235478/relapse-rate-in-survivors-of-acute-autoimmune-thrombotic-thrombocytopenic-purpura-treated-with-or-without-rituximab
#17
Tanja Falter, Stephanie Herold, Veronika Weyer-Elberich, Carina Scheiner, Veronique Schmitt, Charis von Auer, Xavier Messmer, Philipp Wild, Karl J Lackner, Bernhard Lämmle, Inge Scharrer
BACKGROUND:  Autoimmune thrombotic thrombocytopenic purpura (iTTP) is caused by autoantibody-mediated severe a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13) deficiency leading to micro-angiopathic haemolytic anaemia (MAHA) and thrombocytopenia with organ damage. Patients survive with plasma exchange (PEX), fresh frozen plasma replacement and corticosteroid treatment. Anti-CD20 monoclonal antibody rituximab is increasingly used in patients resistant to conventional PEX or relapsing after an acute bout...
October 2018: Thrombosis and Haemostasis
https://read.qxmd.com/read/30072561/autoimmune-cytopenias-and-thrombotic-thrombocytopenic-purpura
#18
Charlotte K Brierley, Sue Pavord
The autoimmune cytopenias are a group of disorders resulting primarily from autoantibody-mediated destruction of blood cells, with variable clinical sequelae depending on the severity and lineage affected. Disease presentation ranges from an asymptomatic finding on a routine full blood count to an acutely unwell patient suffering the clinical consequences of severe anaemia, neutropenia or thrombocytopenia. The cytopenia may be primary or secondary to underlying infectious, immune or malignant processes. Thrombotic thrombocytopenic purpura (TTP) is a distinct, rare but potentially life-threatening entity that classically but not invariably presents with a pentad of acute onset haemolytic anaemia, thrombocytopenia, neurological symptoms, renal impairment and fevers...
August 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://read.qxmd.com/read/30061141/intra-abdominal-ticking-time-bomb-haemosuccus-pancreaticus
#19
Tej Mehta, Oluwagbenga Serrano
A 48-year-old man presented in a near-syncopal state with a 3-day history of melaena, anaemia and epigastric pain. His medical history was significant for chronic autoimmune sclerosing pancreatitis and three previous hospitalisations for enigmatic gastrointestinal bleeding. Symptomatic treatment for anaemia was coupled with oesophagogastroduodenoscopy, two colonoscopies and video capsule endoscopy, but all failed to identify a source of gastrointestinal bleeding. CT angiography of the abdomen revealed extravasation of contrast from a small branch of the splenic artery, later identified as a splenic artery pseudoaneurysm, which was subject to two separate failed embolisation attempts...
July 30, 2018: BMJ Case Reports
https://read.qxmd.com/read/30005859/classification-of-primary-antiphospholipid-syndrome-as-systemic-lupus-erythematosus-analysis-of-a-cohort-of-214-patients
#20
REVIEW
Romain Paule, Nathalie Morel, Véronique Le Guern, Micaela Fredi, Laetitia Coutte, Meriem Belhocine, Luc Mouthon, Claire le Jeunne, Anthony Chauvin, Jean-Charles Piette, Nathalie Costedoat-Chalumeau
OBJECTIVES: To assess the limitations of the SLICC (Systemic Lupus International Collaborating Clinics) classification criteria for systemic lupus erythematosus (SLE), in patients with primary antiphospholipid syndrome (PAPS). METHODS: Retrospective study of a cohort of APS patients (Sydney criteria). We successively excluded patients with (1) at least one "SLE-specific" manifestation (biopsy-proven SLE nephropathy, arthritis, cutaneous, or neurologic SLE manifestations, pericarditis, autoimmune haemolytic anaemia, oral and nasal ulcers, non-scarring alopecia, anti-dsDNA, and anti-Sm antibodies), (2) any other autoimmune connective tissue disease, and/or (3) antinuclear antibodies >1/320...
September 2018: Autoimmunity Reviews
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