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Agranulocytosis

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https://www.readbyqxmd.com/read/28525589/bone-marrow-involvement-in-systemic-lupus-erythematosus
#1
Emilie Chalayer, Nathalie Costedoat-Chalumeau, Odile Beyne-Rauzy, Jacques Ninet, Stephane Durupt, Jacques Tebib, Bouchra Asli, Olivier Lambotte, Martine Ffrench, Christian Vasselon, Pascal Cathébras
BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia, and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a target organ in SLE. Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome...
May 19, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28488730/octreotide-associated-neutropenia
#2
Stacy S Tse, Troy Kish
Drug-induced neutropenia and agranulocytosis are rare adverse events but can be fatal. Neutropenia can be induced by a myriad of drugs from almost every pharmacologic class. Octreotide is a somatostatin analogue that has been used to treat variceal bleeding, acromegaly, and severe diarrhea associated with metastatic tumors, and to reduce symptoms in the setting of malignant bowel obstruction and pseudo-obstruction. The most common adverse effects associated with octreotide include pain at the injection site and gastrointestinal effects such as loose stools, cramping, and nausea; neutropenia is not currently listed as an adverse effect of the drug...
May 10, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28486791/rare-nox3-variants-confer-susceptibility-to-agranulocytosis-during-thyrostatic-treatment-of-graves-disease
#3
T S Plantinga, P Arts, G H Knarren, A H Mulder, I M Wakelkamp, A R Hermus, L A Joosten, M G Netea, P H Bisschop, W W de Herder, H J Beijers, I J de Bruin, C Gilissen, J A Veltman, A Hoischen, J W Smit, R T Netea-Maier
Agranulocytosis is a rare and serious adverse effect of antithyroid drugs, with unknown etiology. The present study aimed to uncover genetic susceptibility and underlying mechanisms of antithyroid drug-induced agranulocytosis (ATDAC). We studied two independent families with familial Graves' disease, of which several members developed ATDAC. In addition, six sporadic ATDAC patients with Graves' disease were investigated. Whole exome sequencing analysis of affected and unaffected family members was performed to identify genetic susceptibility variants for ATDAC, followed by functional characterization of primary granulocytes from patients and unrelated healthy controls...
May 9, 2017: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28448199/deferiprone-for-the-treatment-of-transfusional-iron-overload-in-thalassemia
#4
Janet L Kwiatkowski, Ami Belmont
Transfusional iron overload can lead to hepatic fibrosis, arrhythmias and congestive heart failure and a number of endocrinopathies. Deferiprone is an oral iron chelator recently approved for use in the United States as a second line agent for the treatment of transfusional iron overload in patients with thalassemia. Areas covered: This article will review the data regarding the efficacy of deferiprone for iron chelation and prevention and reversal of iron related complications, the drug's adverse effect profile, and the use of this drug in combination regimens...
April 27, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28437295/clozapine-associated-agranulocytosis-treatment-with-granulocyte-colony-stimulating-factor-granulocyte-macrophage-colony-stimulating-factor-a-systematic-review
#5
John Lally, Steffi Malik, Eromona Whiskey, David M Taylor, Fiona P Gaughran, Amir Krivoy, Robert J Flanagan, Aleksandar Mijovic, James H MacCabe
PURPOSE/BACKGROUND: Clozapine is associated with hematological abnormalities, notably neutropenia, which may progress to agranulocytosis. Granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF) have been used to reduce the frequency and duration of clozapine-associated neutropenia. This review aims to explore the use, efficacy, and tolerability of these cytokines in the treatment of clozapine-associated agranulocytosis. METHODS/PROCEDURES: We conducted a systematic review of published interventional and observational studies, case series, and case reports where G-CSF/GM-CSF was used to treat clozapine-associated agranulocytosis...
April 22, 2017: Journal of Clinical Psychopharmacology
https://www.readbyqxmd.com/read/28418011/more-than-25-years-of-genetic-studies-of-clozapine-induced-agranulocytosis
#6
REVIEW
S A J de With, S L Pulit, W G Staal, R S Kahn, R A Ophoff
Clozapine is one of the most effective atypical antipsychotic drugs prescribed to patients with treatment-resistant schizophrenia. Approximately 1% of patients experience potential life-threatening adverse effects in the form of agranulocytosis, greatly hindering its applicability in clinical practice. The etiology of clozapine-induced agranulocytosis (CIA) remains unclear, but is thought to be a heritable trait. We reviewed the genetic studies of CIA published thus far. One recurrent finding from early candidate gene study to more recent genome-wide analysis is that of the involvement of human leukocyte antigen locus...
April 18, 2017: Pharmacogenomics Journal
https://www.readbyqxmd.com/read/28407835/-recurrent-pulmonary-infection-and-oral-mucosal-ulcer
#7
Fei-Mei Kuang, Lan-Lan Tang, Hui Zhang, Min Xie, Ming-Hua Yang, Liang-Chun Yang, Yan Yu, Li-Zhi Cao
An 8-year-old girl who had experienced intermittent cough and fever over a 3 year period, was admitted after experiencing a recurrence for one month. One year ago the patient experienced a recurrent oral mucosal ulcer. Physical examination showed vitiligo in the skin of the upper right back. Routine blood tests and immune function tests performed in other hospitals had shown normal results. Multiple lung CT scans showed pulmonary infection. The patient had recurrent fever and cough and persistent presence of some lesions after anti-infective therapy...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28405135/severe-bone-marrow-suppression-accompanying-pulmonary-infection-and-hemorrhage-of-the-digestive-tract-associated-with-leflunomide-and-low-dose-methotrexate-combination-therapy
#8
Caihong Qu, Ying Lu, Weimin Liu
A 60-year-old male patient developed hyperpyrexia, cough, expectoration with blood-stained sputum, mouth ulcers, and suppurative tonsillitis after receiving 35 days of combination treatment with leflunomide (LEF) and low-dose methotrexate (MTX) for active rheumatoid arthritis. On admission, routine blood tests showed severe thrombocytopenia, agranulocytosis, and decreased hemoglobin concentration compared with the relatively normal results of 1 month previously during the first hospitalization. Chest radiography revealed inflammation in both lungs, and a fecal occult blood test was positive...
January 2017: Journal of Pharmacology & Pharmacotherapeutics
https://www.readbyqxmd.com/read/28403560/icons-in-paediatrics-rolf-kostmann-1909-1982
#9
Carl Gustaf Ljunggren, Lars Stenhammar, Leif Strömberg
In the medical literature, diseases and syndromes are sometimes named after the first person to publish a report on, or describe, a condition. This is, of course, a great honour, both for the physician and his or her country and only a handful of Swedish medical researchers have achieved this. Swedish paediatrician Rolf Kostmann (Fig. 1) joined this elite group following his report on what he called infantile hereditary agranulocytosis (agranulocytosis infantilis hereditaria), which was first published 1950 in Swedish (1), and his dissertation in 1956 (2), He was the first to describe severe chronic neutropenia as an inherited disease...
April 12, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28392335/graves-disease-in-a-3-year-old-patient-with-agranulocytosis-due-to-anti-thyroid-drugs-radioiodine-ablation-therapy-as-an-effective-alternative
#10
E Espinosa-Muñoz, D Ramírez-Ocaña, A M Martín-García, F J Ruiz-García, C Puentes-Zarzuela
The case is presented of a 3 year-old girl with mitochondrial disease (subacute necrotizing encephalomyelopathy of Leigh syndrome), v-stage chronic kidney disease of a diffuse mesangial sclerosis, as well as developmental disorders, and diagnosed with hyperthyroidism Graves-Basedow disease. Six weeks after starting the treatment with neo-carbimazole, the patient reported a serious case of agranulocytosis. This led to stopping the anti-thyroid drugs, and was treated successfully with (131)I ablation therapy...
April 6, 2017: Revista Española de Medicina Nuclear e Imagen Molecular
https://www.readbyqxmd.com/read/28371438/agranulocytosis-and-mixed-type-autoimmune-hemolytic-anemia-in-primary-sj%C3%A3-gren-s-syndrome-a-case-report-and-review-of-the-literature
#11
Lin Qiao, Jing Chen, Xiao-Mei Leng, Wen Zhang, Bing Han, Yan Zhao, Xiao-Feng Zeng
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. Patients with pSS have a broad spectrum of laboratory features, such as cytopenias and hypergammaglobulinemia. Although hematological abnormalities are usually seen in pSS patients, agranulocytosis and autoimmune hemolytic anemia (AIHA) are rare. Here we describe a 40-year-old woman with pSS who developed both agranulocytosis and mixed-type AIHA. An increased risk of malignancies has also been reported in pSS patients with hematological changes...
December 2016: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28361596/a-case-of-fatal-agranulocytosis-that-developed-in-a-patient-with-%C3%AE-thalassemia-major-treated-with-deferiprone
#12
Maria Mainou, Aggeliki Kotsiafti, Philippos Klonizakis, Vasiliki Soulountsi, Chrysoula Apostolou, Kiriakos Psarras, Efthymia Vlachaki
A 29-year-old male with transfusion-dependent β-thalassemia major (β-TM), splenectomized and on chelation therapy with deferiprone (DFP or L1) due to heart and liver hemosiderosis, presented with high fever and agranulocytosis. Deferiprone was discontinued and a broad spectrum antibiotic therapy was started intravenously. The patient remained febrile and showed no recovery of neutrophil count even after the initiation of granulocyte colony-stimulation factor (G-CSF). After 12 days at the hospital, he developed respiratory failure and was transferred to the intensive care unit (ICU) where he developed multi-organ failure and died 3 days later...
November 2016: Hemoglobin
https://www.readbyqxmd.com/read/28331055/reticular-dysgenesis-international-survey-on-clinical-presentation-transplantation-and-outcome
#13
Manfred Hoenig, Chantal Lagresle-Peyrou, Ulrich Pannicke, Luigi D Notarangelo, Fulvio Porta, Andrew R Gennery, Mary Slatter, Morton J Cowan, Polina Stepensky, Hamoud Al-Mousa, Daifulah Al-Zahrani, Sung-Yun Pai, Waleed Al Herz, Hubert B Gaspar, Paul Veys, Koichi Oshima, Kohsuke Imai, Hiromasa Yabe, Lenora M Noroski, Nico M Wulffraat, Karl-Walter Sykora, Pere Soler-Palacin, Hideki Muramatsu, Mariam Al Hilali, Despina Moshous, Klaus-Michael Debatin, Catharina Schuetz, Eva-Maria Jacobsen, Ansgar S Schulz, Klaus Schwarz, Alain Fischer, Wilhelm Friedrich, Marina Cavazzana
Reticular Dysgenesis (RD) is a rare congenital disorder defined clinically by the combination of severe combined immunodeficiency (SCID), agranulocytosis and sensorineural deafness. Mutations in the gene encoding Adenylate Kinase 2 (AK2) were identified to cause the disorder. Hematopoietic stem cell transplantation (HSCT) is the only option to cure this otherwise fatal disease. Retrospective data on clinical presentation, genetics and outcome of HSCT were collected from centers in Europe, Asia and North America for a total of 32 patients born between 1982 and 2011...
March 22, 2017: Blood
https://www.readbyqxmd.com/read/28325735/rescue-pre-operative-treatment-with-lugol-s-solution-in-uncontrolled-graves-disease
#14
Jan Calissendorff, Henrik Falhammar
BACKGROUND: Graves' disease is a common cause of hyperthyroidism. Three therapies have been used for decades: pharmacologic therapy, surgery and radioiodine. In case of adverse events, especially agranulocytosis or hepatotoxicity, pre-treatment with Lugol's solution containing iodine/potassium iodide to induce euthyroidism before surgery could be advocated, but this has rarely been reported. METHODS: All patients hospitalised due to uncontrolled hyperthyroidism at the Karolinska University Hospital 2005-2015 and treated with Lugol's solution were included...
May 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28322460/dapsone-induced-agranulocytosis-possible-involvement-of-low-activity-n-acetyltransferase-2
#15
Ines Potočnjak, Robert Likić, Iveta Šimić, Danica Juričić Nahal, Ivana Čegec, Lana Ganoci, Nada Božina
Dapsone-induced agranulocytosis is a rare but potentially fatal adverse drug reaction (ADR). A 45-year old male Caucasian patient developed agranulocytosis caused by dapsone (diamino-diphenyl sulfone) which he was prescribed for leukocytoclastic vasculitis. Patient's treatment consisted of termination of dapsone, antibiotic therapy, and granulocyte colony stimulating factor leading to prompt improvement of symptoms and normalization of laboratory blood values. Diagnostic evaluation revealed methemoglobinemia and excluded glucose-6-phosphate dehydrogenase (G6PD) deficiency...
March 21, 2017: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28320470/methimazole-associated-eosinophilic-pleural-effusion-a-case-report
#16
Pedro Gaspar-da-Costa, Filipa Duarte Silva, Júlia Henriques, Sónia do Vale, Sandra Braz, João Meneses Santos, Rui M M Victorino
BACKGROUND: Adverse reactions associated to anti-thyroid drugs include fever, rash, arthralgia, agranulocytosis and hepatitis that are thought to be hypersensitivity reactions. Five cases of pleural effusion associated to thionamides have also been reported, two with propylthiouracil and three with carbimazole. CASE PRESENTATION: We report here a case of a 75-year-old man admitted because of unilateral pleural effusion. The patient had a recent diagnosis of hyperthyroidism and 6 days after starting methimazole complained of pleuritic chest pain...
March 21, 2017: BMC Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28315874/multiple-drug-hypersensitivity
#17
Werner J Pichler, Yuttana Srinoulprasert, James Yun, Oliver Hausmann
Multiple drug hypersensitivity (MDH) is a syndrome that develops as a consequence of massive T-cell stimulations and is characterized by long-lasting drug hypersensitivity reactions (DHR) to different drugs. The initial symptoms are mostly severe exanthems or drug rash with eosinophilia and systemic symptoms (DRESS). Subsequent symptoms due to another drug often appear in the following weeks, overlapping with the first DHR, or months to years later after resolution of the initial presentation. The second DHR includes exanthema, erythroderma, DRESS, Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), hepatitis, and agranulocytosis...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28314885/adverse-effects-of-levamisole-in-cocaine-users-a-review-and-risk-assessment
#18
REVIEW
Tibor Markus Brunt, Jorrit van den Berg, Ed Pennings, Bastiaan Venhuis
The immunomodulatory adjuvant and antihelminth levamisole is increasingly used as an adulterant in cocaine worldwide. An accumulating body of clinical and toxicological literature has appeared since 2010 describing neutropenia, agranulocytosis, leukoencephalopathy and vasculitis in cases associated with levamisole-adulterated cocaine. Mostly, neutropenia and agranulocytosis were reported, characterized by a decimation of neutrophils. A large proportion of cases also involved vasculopathy, characterized by pronounced black and purple skin purpura with cutaneous necrosis...
March 17, 2017: Archives of Toxicology
https://www.readbyqxmd.com/read/28291036/potential-mechanisms-of-hematological-adverse-drug-reactions-in-patients-receiving-clozapine-in-combination-with-proton-pump-inhibitors
#19
Michał Wiciński, Mateusz M Węclewicz, Mateusz Miętkiewicz, Bartosz Malinowski, Elżbieta Grześk, Joanna Klonowska
Clozapine is a second-generation antipsychotic which has proven efficacy in treating the symptoms of schizophrenia. Although clozapine therapy is associated with a number of adverse drug reactions, it is frequently used. One of the most common adverse drug reactions is gastroesophageal reflux disease which is an indication for treatment with proton pump inhibitors (PPIs). Coadministration of clozapine and PPIs increases the risk of hematological adverse drug reactions, including neutropenia and agranulocytosis...
March 2017: Journal of Psychiatric Practice
https://www.readbyqxmd.com/read/28276328/chronic-use-of-metamizole-not-so-safe-after-all
#20
F E R Vuik, P Koehestanie, A H E Herbers, J S Terhaar Sive Droste
Metamizole can be used in both short- and long-term pain relief therapies and has a relatively favourable safety profile compared with classic NSAIDs. Metamizole is also infamous because of its potential fatal adverse drug reaction, agranulocytosis. Although this risk varies, it is estimated to occur in less than one million metamizole prescriptions. We describe a case of a 68-year-old patient who developed leukopenia after using metamizole.
March 2017: Netherlands Journal of Medicine
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