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Autoimmune Anaemia

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https://www.readbyqxmd.com/read/30072561/autoimmune-cytopenias-and-thrombotic-thrombocytopenic-purpura
#1
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://www.readbyqxmd.com/read/30061141/intra-abdominal-ticking-time-bomb-haemosuccus-pancreaticus
#2
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://www.readbyqxmd.com/read/30005859/classification-of-primary-antiphospholipid-syndrome-as-systemic-lupus-erythematosus-analysis-of-a-cohort-of-214-patients
#3
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...
July 10, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/30005273/non-genetic-and-genetic-risk-factors-for-adult-cerebral-venous-thrombosis
#4
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...
July 6, 2018: Thrombosis Research
https://www.readbyqxmd.com/read/29991541/cold-agglutinin-mediated-autoimmune-haemolytic-anaemia-associated-with-diffuse-large-b-cell-lymphoma
#5
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
https://www.readbyqxmd.com/read/29982182/igg4-related-disease-coexisting-with-autoimmune-haemolytic-anaemia
#6
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
https://www.readbyqxmd.com/read/29979258/autoimmune-haemolytic-anaemia-and-autoimmune-thrombocytopenia-in-childhood-onset-systemic-lupus-erythematosus-updates-on-pathogenesis-and-treatment
#7
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
https://www.readbyqxmd.com/read/29948002/lupus-retinopathy-a-marker-of-active-systemic-lupus-erythematosus
#8
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
https://www.readbyqxmd.com/read/29869203/fostamatinib-first-global-approval
#9
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
https://www.readbyqxmd.com/read/29866698/cutaneous-nocardiosis-by-a-new-pathogenic-species-nocardia-grenadensis
#10
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
https://www.readbyqxmd.com/read/29804073/bronchiolitis-obliterans-organising-pneumonia-as-an-initial-manifestation-in-a-patient-with-systemic-lupus-erythematosus-a-rare-presentation
#11
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
https://www.readbyqxmd.com/read/29757134/second-line-therapy-in-paediatric-warm-autoimmune-haemolytic-anaemia-guidelines-from-the-associazione-italiana-onco-ematologia-pediatrica-aieop
#12
Saverio Ladogana, Matteo Maruzzi, Piera Samperi, Annalisa Condorelli, Maddalena Casale, Paola Giordano, Lucia D Notarangelo, Piero Farruggia, Fiorina Giona, Agostino Nocerino, Silvia Fasoli, Maria L Casciana, Maurizio Miano, Fabio Tucci, Tommaso Casini, Paola Saracco, Wilma Barcellini, Alberto Zanella, Silverio Perrotta, Giovanna Russo
No abstract text is available yet for this article.
July 2018: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/29737533/a-randomized-first-in-human-healthy-volunteer-trial-of-sutimlimab-a-humanized-antibody-for-the-specific-inhibition-of-the-classical-complement-pathway
#13
Johann Bartko, Christian Schoergenhofer, Michael Schwameis, Christa Firbas, Martin Beliveau, Colin Chang, Jean-Francois Marier, Darrell Nix, James C Gilbert, Sandip Panicker, Bernd Jilma
Aberrant activation of the classical complement pathway is the common underlying pathophysiology of orphan diseases such as bullous pemphigoid, antibody-mediated rejection of organ transplants, cold agglutinin disease, and warm autoimmune hemolytic anemia. Therapeutic options for these complement-mediated disorders are limited and sutimlimab, a humanized monoclonal antibody directed against complement factor C1s, may be potentially useful for inhibition of the classical complement pathway. A phase I, first-in-human, double-blind, randomized, placebo-controlled, dose-escalation trial of single and multiple doses of sutimlimab or placebo was conducted in 64 volunteers to evaluate safety, tolerability, pharmacokinetic, and pharmacodynamic profiles...
May 8, 2018: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/29732532/pathogenesis-diagnosis-and-treatment-of-anaemia-in-immune-mediated-gastrointestinal-disorders
#14
REVIEW
Gaetano Bergamaschi, Antonio Di Sabatino, Gino R Corazza
Immune-mediated disorders affecting the gastrointestinal (GI) tract may compromise GI integrity, interfere with the absorption of nutrients and cause bleeding and inflammation. All these features contribute to the pathogenesis of anaemia, the most prevalent extra-intestinal manifestation of immune-mediated GI disorders. Anaemia is most commonly due to iron deficiency and/or inflammation, but vitamin deficiencies and, more infrequently, autoimmune haemolysis or drug-induced myelosuppression can be involved. Here we address several issues related to the differential diagnosis and treatment of anaemia in immune-mediated GI disorders, giving particular relevance to the problem of iron deficiency anaemia associated with inflammation...
May 6, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29688999/should-giant-cell-hepatitis-with-autoimmune-haemolythic-anaemia-be-considered-a-paediatric-autoimmune-liver-disease
#15
Giorgina Mieli-Vergani, Diego Vergani
No abstract text is available yet for this article.
May 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29669775/a-case-of-autoimmune-haemolytic-anaemia-after-39-cycles-of-nivolumab
#16
Hira Shaikh, Nour Daboul, Mary Albrethsen, Salman Fazal
With growing use of nivolumab, rare but serious side effects have surfaced in some patients. We present a case of autoimmune haemolytic anaemia that developed after 39 cycles of nivolumab. A 78-year-old man with metastatic lung adenocarcinoma, refractory to multiple lines of chemotherapy was switched to nivolumab. After around 2 years of stable course on nivolumab, he developed transfusion-dependent anaemia with haemoglobin of 8.6 g/dL. Nivolumab was held immediately. Bone marrow biopsy findings were inconclusive of myelodysplastic syndrome...
April 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29669774/transient-immune-mediated-agranulocytosis-following-mycoplasma-pneumoniae-infection
#17
Luani Barge, Gail Pahn, Nicholas Weber
Mycoplasma pneumoniae is a common respiratory pathogen which may cause haematological manifestations including haemolytic anaemia and thrombocytopaenia. Severe neutropaenia is rare with very few cases reported in the literature. An 85-year-old man was transferred to our facility with agranulocytosis in the context of an infective exacerbation of chronic obstructive pulmonary disease with positive serological testing for M. pneumoniae. No alternative infective, autoimmune or lymphoproliferative cause of the neutropaenia was identified...
April 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29626026/dilated-cardiomyopathy-as-the-first-presentation-of-coeliac-disease-association-or-causation
#18
Peysh Patel, Fraser Smith, Niamh Kilcullen, Nigel Artis
Global ventricular impairment is a frequent presentation in clinical practice, but dissection of causative mechanisms from clinical associations is challenging. We present the case of a 19-year-old man who presented with dilated cardiomyopathy as the first presentation of coeliac disease. The manifestation of iron deficiency anaemia prompted gastroenterology input and enabled accurate diagnosis. It is unclear whether coeliac disease was simply coexistent or directly implicated in pathophysiology. Mechanisms may relate to nutritional deficiencies or autoimmune myocarditis arising from cross-reactivity...
March 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29599802/tuberculosis-induced-autoimmune-haemolytic-anaemia-a-systematic-review-to-find-out-common-clinical-presentations-investigation-findings-and-the-treatment-options
#19
REVIEW
Devarajan Rathish, Sisira Siribaddana
Background: Tuberculosis induced autoimmune haemolytic anaemia is a rare entity. The aim of this study was to explore its common presentations, investigation findings and treatment options through a systematic review of published reports. Methods: PubMed, Trip, Google Scholar, Science Direct, Cochrane Library, Open-Grey, Grey literature report and the reference lists of the selected articles were searched for case reports in English on tuberculosis induced auto-immune haemolytic anaemia...
2018: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/29574435/autoimmune-pancreatitis-with-concomitant-autoimmune-haemolytic-anaemia
#20
Lauren Passby, Matthew Harris, Ahmed Al-Mukhtar
Autoimmune pancreatitis (AIP) is an infrequent cause of acute pancreatitis, being more commonly associated with chronic pancreatitis. AIP can be associated with other autoimmune manifestations, including Sjögren's, inflammatory bowel disease, primary biliary cirrhosis, rheumatoid arthritis, hypothyroidism and sarcoidosis. Rarely, concurrent autoimmune haemolytic anaemia (AIHA) is observed, as seen in our case report of a 33-year-old postpartum woman.
March 23, 2018: BMJ Case Reports
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