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Autoimmune Neutropenia

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...
October 5, 2018: 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...
September 21, 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
Ariane Barbacki, Peter G Maliha, Marie Hudson, David Small
Immune checkpoint inhibitors have revolutionized cancer therapy. Given their mechanism of action, immune-related adverse events have been associated with their use. We present the first documented case of pembrolizumab-induced grade IV neutropenia. A 73-year-old women known for myositis, Crohn's disease, and hypothyroidism and diagnosed with PD-L1 positive stage IV pulmonary adenocarcinoma is treated with Pembrolizumab. She develops grade IV neutropenia 2 weeks after her second infusion. She is therefore hospitalized and treated initially with corticosteroids, granulocyte colony-stimulating factor, and intravenous immunoglobulins...
September 2018: Anti-cancer Drugs
Barbara Hauck-Dlimi, Mohammed Damrah, Susanne Achenbach, Stephan Ott, Robert Zimmermann, Juergen Zingsem, Julian Strobel
BACKGROUND: Human neutrophil antigens (HNA) are able to provoke allo- and autoimmune antibodies which lead to reactions like autoimmune and neonatal neutropenia. However, until now no data about HNA genotype distribution in Syrian population exists. The aim of this study was to determine the HNA allele frequencies in the largest group asking for asylum in Germany since 2015. Allele frequencies were compared to data from German blood donors. Therefore, we calculated the risk of alloimmunization and associated transfusion reactions, as well as the risk of developing neonatal neutropenia for newborns of mixed race couples...
April 1, 2018: Clinical Laboratory
James Elliott, Elizabeth Villiers
In humans, large granular lymphocytic leukaemia (LGLL) is a low-grade, indolent lymphoproliferative disorder of large granular lymphocytes (LGL) associated with autoimmune disorders; including rheumatoid arthritis and single or multiple cytopenias; particularly neutropenia. Therapy largely centres around immunosuppression which aims to resolve the immune-mediated secondary pathology, often without eradicating the neoplastic clone. The most effective agents appear to be cyclophosphamide, cyclosporine and methotrexate...
2018: Open Veterinary Journal
Ara C Almécija, Vanesa Pérez, María Baro, Pilar Guerra-García, Jose L Vivanco
Kabuki syndrome is a rare genetic disorder characterized by congenital anomalies and developmental delay. It is often associated with impaired immune response and autoimmune abnormalities. We report the clinical case of a girl with Kabuki syndrome who developed autoimmune neutropenia, not previously reported, followed by hemolytic anemia and autoimmune thrombocytopenia.
April 20, 2018: Journal of Pediatric Hematology/oncology
Safi Alqatari, Peter Riddell, Sinead Harney, Michael Henry, Grainne Murphy
BACKGROUND: Clinically hypomyopathic dermatomyositis is a rare disease that is important to recognize, investigate and treat early as it is associated with poor prognosis. In a proportion of patients, myositis specific antibodies could be negative, but with high clinical suspicion, myositis associated antibodies should be ordered. Anti-MDA-5 antibodies was reported in literature to be associated with severe and rapidly progressive interstitial lung disease, with few case reports of pneumothorax and/or pneumomediastinum...
April 17, 2018: BMC Pulmonary Medicine
Masahiro Tokunaga, Masahide Yamada, Atsushi Kondo, Mai Mishima, Shinichi Yoshikawa, Shinya Inoue, Takako Morita, Nobuhiko Tominaga
Adult-onset autoimmune neutropenia (AIN) is rarely self-limiting, unlike infant-onset AIN. Although several therapeutic agents have been reported, including corticosteroids, more effective treatment options may exist. Here, we describe neutrophil recovery by eltrombopag in a 52-year-old male AIN patient with immune thrombocytopenia (ITP) who was referred to our hospital with severe neutropenia. Within a year of referral, he developed moderate thrombocytopenia. He was diagnosed with AIN and concurrent ITP, based on the detection of antineutrophil antibodies and bone marrow aspiration, respectively...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Mari Asakura, Toshiyuki Tanaka, Kensuke Shoji, Shuhei Karakawa, Akira Ishiguro, Isao Miyairi
BACKGROUND: Suppurative cervical lymphadenitis is rare in children with limited information about the frequency of neutropenia in any series. METHODS: We conducted a retrospective review of pediatric patients in whom suppurative cervical lymphadenitis was diagnosed between April 2011 and March 2016 at a tertiary children's hospital in Tokyo, Japan. Microbiologically confirmed cases of cervical lymphadenitis with abscess formation were included in the analysis. RESULTS: Twenty-five patients (median age: 1 year old, 64% female) with abscess formation due to Staphylococcus aureus were identified...
April 2, 2018: Pediatric Infectious Disease Journal
Yayoi Shimazu, Tatsuki Uchiyama, Chisaki Mizumoto, Tomoharu Takeoka, Masaaki Tsuji, Kenjiro Tomo, Koji Takaori, Naoki Sakai, Tomoko Okuno, Tatsuharu Ohno
IgG4-related disease (IgG4RD) is a multi-organ disorder characterized by an elevated serum IgG4 level and IgG4-positive plasma cell infiltration of the affected organs, accompanied by tissue fibrosis and sclerosis. Although it can affect any organ, to our knowledge, no cases involving concurrent autoimmune neutropenia and thrombocytopenia have been reported. A 62-year-old man visited our hospital and was diagnosed with IgG4RD accompanied by interstitial pneumonitis, lymphadenopathy, and interstitial nephritis...
July 1, 2018: Internal Medicine
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