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Autoimmune Anaemia

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
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
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
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
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
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
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
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
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
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
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
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
Mackenzie Green, Toby Styles, Timothy Russell, Charif Sada, Ebrima Jallow, Jack Stewart, Otar Lazariashvili, Irina Lubomirova, Ioana Cotlarciuc, Sapna Sharma, Thang S Han, Pankaj Sharma
INTRODUCTION: A wide variety of non-genetic and genetic factors have been shown to associate with increased risk for cerebral venous thrombosis (CVT). However, there is a paucity of risk factor data and conclusions about their impact are often conflicting. Herein, we quantified the associations of non-genetic and genetic risk factors for CVT in adults. MATERIALS AND METHODS: Electronic databases were searched up to January 2017. Meta-analyses were performed (RevMan v5...
September 2018: Thrombosis Research
Sariya Wongsaengsak, Magdalena Czader, Attaya Suvannasankha
Cold agglutinin-mediated autoimmune haemolytic anaemia is associated with the development of autoantibodies that can agglutinate red blood cells at cold temperatures. While primary cold agglutinin disease is an idiopathic lymphoproliferative disorder, secondary cold agglutinin syndrome (CAS) complicates other diseases such as infections, autoimmune diseases and cancers, mostly low-grade lymphomas. Early recognition, treatment of CAS and treatment of its associated underlying diseases are crucial to a successful outcome...
July 10, 2018: BMJ Case Reports
Kai-Chun Wang, Hsien-Tzung Liao, Chang-Youh Tsai
An 85-year-old man presented with a pale appearance and generalised pruritic papules. Laboratory investigations disclosed eosinophilia, autoimmune haemolytic anaemia, mixed hyperbilirubinaemia, cholestasis and elevated serum IgG4 levels. Abdominal sonography and CT showed progressive dilatation of biliary trees, with diffuse pancreatic enlargement and a subtle capsule-like low-density rim around the pancreatic head and body. Endoscopic retrograde cholangiopancreatography found no stone-related biliary obstruction, while endoscopic transpapillary biopsy demonstrated chronic inflammation only...
July 6, 2018: BMJ Case Reports
Jessica Neely, Emily von Scheven
PURPOSE OF REVIEW: Autoimmune haemolytic anaemia (AIHA) and autoimmune thrombocytopenia are common complications of childhood-onset lupus, which may be life-threatening. A greater understanding of the pathogenesis of these haematologic manifestations will enhance our understanding of the biology of systemic lupus erythematosus (SLE) and inform the identification of novel treatments. RECENT FINDINGS: The mechanisms underlying AIHA and autoimmune thrombocytopenia are incompletely understood and likely multifactorial...
September 2018: Current Opinion in Rheumatology
Gaurav Seth, K G Chengappa, Durga Prasanna Misra, Ramesh Babu, Pooja Belani, K C Shanoj, Gunjan Kumar, Vir Singh Negi
Retinopathy in the context of systemic lupus erythematosus (SLE) is associated with severe disease and poorer prognosis. We studied retinopathy in our cohort of Indian lupus patients. Four hundred and thirty-seven patients fulfilling the Systemic Lupus International Collaborating Clinics-American College of Rheumatology-2012 criteria, attending the department of Clinical Immunology were enrolled under this cross-sectional study. A comprehensive clinical (including ophthalmological) examination and immunological profile were performed...
August 2018: Rheumatology International
Anthony Markham
Rigel Pharmaceuticals are developing the spleen tyrosine kinase (SYK) inhibitor fostamatinib (TAVALISSE™) as a treatment for immune thrombocytopenia (ITP), autoimmune haemolytic anaemia and IgA nephropathy. Based on positive results in the phase III FIT clinical trial program, the drug was recently approved in the US as a treatment for thrombocytopenia in adult patients with chronic ITP who have had an insufficient response to a previous treatment. This article summarizes the milestones in the development of fostamatinib leading to this first approval...
June 2018: Drugs
Rui Pedro Santos, Juliana Almeida, Filipa Tavares Almeida, Maria da Luz Duarte
Nocardiosis is a rare, predominantly opportunistic, suppurative disease caused by bacteria of the order Actinomycetales. There are currently more than 100 species of Nocardia described, less than half are pathogenic to humans. Cutaneous nocardiosis can be caused by direct inoculation from a contaminated material or by secondary dissemination. The authors present a 70-year-old man with an autoimmune haemolytic anaemia treated with prednisolone and azathioprine. The patient presented multiple erythematous tender nodules with linear distribution and proximal progression along the left upper limb with 2 months of evolution...
June 4, 2018: BMJ Case Reports
Shraddha Jatwani, Richa Handa, Karan Jatwani, Karan Chugh
Bronchiolitis obliterans organising pneumonia as an initial manifestation of systemic lupus erythematosus (SLE) is a rare and uncommon presentation. We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion. Work-up suggested a diagnosis of active SLE with anaemia, thrombocytopenia, positive antinuclear antibodies (ANAs) and positive anti-double-stranded DNA. On retrospective review of patient records, from 8 years prior to presentation, lung biopsy histology consistent with bronchiolitis obliterans organising pneumonia with positive ANA serology was found, without any further autoimmune work-up...
May 26, 2018: BMJ Case Reports
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