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Autoimmune Neutropenia

Nicola Kleine-Eggebrecht, Christian Staufner, Simone Kathemann, Magdeldin Elgizouli, Robert Kopajtich, Holger Prokisch, Elke Lainka
Pediatric intractable autoimmune hepatitis is rare and may be responsible for acute liver failure. Mutations in the itchy E3 ubiquitin protein ligase ( ITCH ) gene (located on chromosome 20q11.22) can lead to a deficiency of the encoded protein, resulting in increased T-cell activity with lack of immune tolerance and manifestation of a complex systemic autoimmune disease. A 1-year-old girl of consanguineous parents received a liver transplant (LT) because of acute liver failure attributed to a drug-induced hypereosinophilic syndrome with positive liver-kidney-mikrosome-2 antibodies...
January 31, 2019: Pediatrics
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
Wilma Barcellini, Bruno Fattizzo, Agostino Cortelezzi
The physiology of the immune system involves morphologic and functional changes occurring along ageing, with a decrease in immune response and an increase in autoimmune phenomena, even in the absence of overt disese. Autoimmune cytopenias, namely autoimmune hemolytic anemia (AIHA), chronic idiopathic neutropenia (CIN) and aplastic anemia (AA), show different epidemiologic predilection, but are increasingly diagnosed in the elderly, where complications and comorbidities are more frequent. A systematic review of recent literature, shows that comorbidities as well as underlying deficiencies, medications, neoplasms, and, pathophysiologic chronic organ failures, frequently challenge the differential diagnosis in this setting and should always be evaluated and excluded...
December 2018: European Journal of Internal Medicine
Brigitte Katharina Flesch, Angelika Reil
Background and Objective: Antibodies to human neutrophil antigens (HNAs) have been implicated in transfusion-related acute lung injury and allo- and autoimmune neutropenia. To date, five HNA systems are assigned, and during the last decades enormous efforts have been undertaken to identify the underlying genes and to characterize the antigens. This review of the literature will provide the current genetic, molecular and functional information on HNAs. Recent Findings: New information on alleles and antigens has been added to nearly each of the five HNA systems...
October 2018: Transfusion Medicine and Hemotherapy
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
Piero Farruggia, Francesca Fioredda, Giuseppe Puccio, Daniela Onofrillo, Giovanna Russo, Angelica Barone, Sonia Bonanomi, Gianluca Boscarol, Andrea Finocchi, Roberta Ghilardi, Paola Giordano, Saverio Ladogana, Giuseppe Lassandro, Laura Luti, Tiziana Lanza, Rosalba Mandaglio, Nicoletta Marra, Baldassare Martire, Elena Mastrodicasa, Milena Motta, Lucia Dora Notarangelo, Marta Pillon, Laura Porretti, Jessica Serafinelli, Angela Trizzino, Fabio Tucci, Marinella Veltroni, Federico Verzegnassi, Ugo Ramenghi, Carlo Dufour
Autoimmune neutropenia of infancy (AIN) is characterized by low risk of severe infection, tendency to spontaneously resolve and typically onset at < 4-5 years of age; it is due to auto-antibodies whose detection is often difficult. In case of negativity of 4 anti-neutrophils autoantibody tests, after having excluded ethnic, post infection, drug induced or congenital neutropenia, according to the Italian guidelines the patients will be defined as affected by "idiopathic neutropenia" (IN). We describe the characteristics of 85 IN patients enrolled in the Italian neutropenia registry: they were compared to 336 children affected by AIN...
November 19, 2018: American Journal of Hematology
Ruo-Lan Gong, Jing Wu, Tong-Xin Chen
Objectives: Severe congenital neutropenia (SCN) is a primary immunodeficiency disease characterized by the early onset of recurrent infections and persistent severe neutropenia, with or without genetic defect. We aimed to study the different clinical features and hematological and bone marrow characteristics of patients with SCN and the non-congenital form of severe neutropenia (SN) with unknown etiology. Methods: Thirty-nine Chinese children with severe neutropenia for longer than 6 months unrelated to virus infection or autoimmune diseases were enrolled in the study to analyse the clinical, laboratory, and molecular characteristics...
2018: Frontiers in Pediatrics
Marie Warny, Jens Helby, Børge Grønne Nordestgaard, Henrik Birgens, Stig Egil Bojesen
BACKGROUND: Neutropenia increases the risk of infection, but it is unknown if this also applies to lymphopenia. We therefore tested the hypotheses that lymphopenia is associated with increased risk of infection and infection-related death in the general population. METHODS AND FINDINGS: Of the invited 220,424 individuals, 99,191 attended examination. We analyzed 98,344 individuals from the Copenhagen General Population Study (Denmark), examined from November 25, 2003, to July 9, 2013, and with available blood lymphocyte count at date of examination...
November 2018: PLoS Medicine
Carly C Dixon, Francesca J Thomson, Siobhan M Barry
Autoimmune neutropenia is a self-limiting condition characterized by the presence of granulocyte-specific autoantibodies. This case report describes a 2-year-old female who presented to the Department of Pediatric Dentistry with a history of oral ulceration, complicated by high temperature, gingival hypertrophy and gingival hemorrhage. These symptoms had precipitated an admission to a district general hospital and diagnosis of viral tonsillitis. The severity of the presentation to the Pediatric Dental Department prompted a review of recent discharge blood investigations, which revealed a neutrophil count of 0...
2019: Journal of Clinical Pediatric Dentistry
N Suzanne Emil, David R Cisneros, Santhi Penmetsa, Joshua H Duchesne, Wilmer L Sibbitt, James I Gibb, Leonard E Noronha, Monthida Fangtham, Roderick A Fields, Arthur D Bankhurst
BACKGROUND/OBJECTIVE: Immunostimulatory drugs including immune checkpoint inhibitors and levamisole can induce inflammatory disease including vasculitis, rashes, tissue necrosis, and arthritis. METHODS: This prospective cohort study determined the 5-year outcomes of cocaine-levamisole-induced inflammatory disease as to outcomes and survival. Thirty-one consecutive cocaine-levamisole autoimmune patients and 45 primary vasculitis patients were characterized as to clinical differentiating features, antineutrophil cytoplasmic antibody (ANCA) status, treatment, the presence of acute and chronic arthritis, and 5-year outcome...
September 27, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Fabrice Barlesi, Johan Vansteenkiste, David Spigel, Hidenobu Ishii, Marina Garassino, Filippo de Marinis, Mustafa Özgüroğlu, Aleksandra Szczesna, Andreas Polychronis, Ruchan Uslu, Maciej Krzakowski, Jong-Seok Lee, Luana Calabrò, Osvaldo Arén Frontera, Barbara Ellers-Lenz, Marcis Bajars, Mary Ruisi, Keunchil Park
BACKGROUND: Antibodies targeting the immune checkpoint molecules PD-1 or PD-L1 have demonstrated clinical efficacy in patients with metastatic non-small-cell lung cancer (NSCLC). In this trial we investigated the efficacy and safety of avelumab, an anti-PD-L1 antibody, in patients with NSCLC who had already received platinum-based therapy. METHODS: JAVELIN Lung 200 was a multicentre, open-label, randomised, phase 3 trial at 173 hospitals and cancer treatment centres in 31 countries...
November 2018: Lancet Oncology
Feng Huang, Zu-Chun Luo
Several recently published clinical trials have shown tofacitinib to be effective in the treatment of autoimmune diseases. This drug is commonly prescribed either in a 5-mg or in a10-mg dosage twice daily. In this review, we aimed to systematically compare the adverse drug events which were observed with 5 mg versus 10 mg tofacitinib for the treatment of autoimmune diseases. MEDLINE, EMBASE, the Cochrane library, and were searched (from March to April 2018) for suitable English publications (published before April 2018)...
September 22, 2018: Clinical Rheumatology
Aline Moignet, Thierry Lamy
Large granular lymphocyte (LGL) leukemia has been recognized in the World Health Organization classifications among mature T cell and natural killer cell neoplasms and is divided into three categories. Chronic T cell leukemia and natural killer cell lymphocytosis can be considered as a similar spectrum of an indolent disease characterized by cytopenias and autoimmune conditions. The last category, aggressive natural killer cell LGL leukemia is very rare, related to Epstein-Barr virus, and seen mainly in young Asian people...
May 23, 2018: American Society of Clinical Oncology Educational Book
Jaroslav Čermák
Granulocytopenia is defined as a decrease of peripheral blood granulocytes below lower limit of normal range. Patients with severe granulocytopenia - agranulocytosis exhibit < 0.5 × 109/l granulocytes in peipheral blood. Granulocytopenia may result from congenital or acquired defective production of granulocyte precursors or it may be a consequence of increased destruction of mature granulocytes, most frequently caused by immune mechanisms. Investigation of origin of granulocytopenia must be connected with exclusion of etiological agents causing secondary neutropenia (infections, autoimmune disorders, drugs, LGL syndrome)...
2018: Vnitr̆ní Lékar̆ství
A Kumar Arun, Anandan Senthamizhselvi, Suresh Hemamalini, Eunice S Edison, Anu Korula, N A Fouzia, Biju George, Vikram Mathews, Poonkuzhali Balasubramanian
AIMS: Congenital and cyclical neutropenia are rare inherited diseases that result in recurrent life-threatening bacterial infections due to a deficiency of mature neutrophils. Cyclical neutropenia is usually caused by heterozygous ELANE mutations while congenital neutropenia is genetically heterogeneous with mutations in genes like ELANE, HAX-1, G6PC3 and GFI1. The presence of ELANE mutation aids in the establishment of diagnosis and rules out other secondary causes of neutropenia such as autoimmune cytopenia and evolving aplasia...
August 31, 2018: Journal of Clinical Pathology
Sanket Shah, Chengappa G Kavadichanda, Pooja Belani, Rajesh N Ganesh, Vir Singh Negi
Biologics have emerged as an important modality of treatment in rheumatic diseases and have allowed the rheumatologist to explore varied therapeutic uses of these drugs. Rituximab, a monoclonal antibody against CD20 receptor is an important member of the biologic armamentarium for the treatment of various refractory autoimmune inflammatory rheumatic diseases. The drug is now widely used in systemic lupus erythematosus for several complications which are refractory to conventional therapy. Although relatively safe, the post-marketing surveillance of rituximab has revealed a few rare but important adverse reactions...
August 30, 2018: International Journal of Rheumatic Diseases
Marta Lis-Sochocka, Patrycja Chylińska-Wrzos, Ewelina Wawryk-Gawda, Barbara Jodłowska-Jędrych
BACKGROUND: Cladribine is a useful immunosuppressive drug for the treatment of autoimmune diseases, leukemias and multiple sclerosis (MS). Despite the drug having low toxicity, side effects have been reported connected with myelosuppression, neutropenia and severe anemia. OBJECTIVES: The objective of this study was to investigate the influence of cladribine on lung pathomorphology and the expression of caspase 1 using immunohistochemistry method. MATERIAL AND METHODS: The study was conducted on Wistar rats, which were divided into: one control group (C) and one experimental group (E)...
August 6, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Yasuhisa Yokoyama, Akiyoshi Takami, Yasuo Mori, Shinya Sato, Takahiro Suzuki, Masahiko Gosho, Naoshi Obara, Yasushi Miyazaki, Koichi Akashi, Shigeru Chiba
Chronic neutropenia is a rare hematological entity characterized by isolated neutropenia without anemia or thrombocytopenia. The diagnostic criteria and classification of chronic neutropenia have been more elusive in adult cases than pediatric cases. We herein propose the Reference Guide for Adult Chronic Neutropenia. In this guide, diagnosis of chronic neutropenia is based on recurrent or continuous neutropenia defined by absolute neutrophil count (ANC) less than 1,500/µl for over three or more months. Severity of chronic neutropenia is defined as follows: severe, ANC <500/µl; moderate, ANC between 500 and 1,000/µl; mild, ANC between 1,000 and 1,500/µl...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
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
Tim R De Back, Arnon P Kater, Sanne H Tonino
Chronic lymphocytic leukemia (CLL) is frequently complicated by cytopenias, either due to bone marrow infiltration or autoimmunity, resulting in autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), pure red cell aplasia (PRCA), or autoimmune neutropenia (AIN). Morbidity due to autoimmune cytopenias (AIC) can be substantial; in addition, infection risk increases and pre-existing infections might deteriorate due to immunosuppressive medication. In the aging population, CLL occurs more frequently and AIC related to CLL represent a growing clinical challenge...
August 2018: Expert Review of Hematology
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