scholarly journals Immune neutropenia mediated by micafungin

2019 ◽  
Author(s):  
Amir H. Ameri ◽  
Brian R. Curtis ◽  
David B. Sykes
Keyword(s):  
Immune Neutropenia

Evans syndrome with auto‐immune neutropenia

2021 ◽  
Author(s):  
Mariya MA Bilochvostenko ◽  
Aton AAM Ermens ◽  
Roel RB Fiets
Keyword(s):  
Evans Syndrome ◽  
Immune Neutropenia

The severity of immune neutropenia correlates with the maturational specificity of antineutrophil antibodies

1984 ◽  
Vol 58 (2) ◽  
pp. 209-215 ◽  
Author(s):  
David C. Harmon ◽  
Sigmund A. Weitzman ◽  
Thomas P. Stossel
Keyword(s):  
Immune Neutropenia

scholarly journals Intracranial Haemorrhage as a Fatal Complication of Evans Syndrome: A Case Report

2019 ◽  
Vol 2 (2) ◽  
pp. 66-69
Author(s):  
Olita Shilpakar ◽  
Bibek Rajbhandari ◽  
Bipin Karki ◽  
Umesh Bogati
Keyword(s):  
Intracranial Haemorrhage ◽  
Early Recognition ◽  
Clinical Manifestations ◽  
Health Care Facilities ◽  
Evans Syndrome ◽  
Fatal Complication ◽  
Hematologic Disorder ◽  
Immune Mediated ◽  
Life Threatening ◽  
Immune Neutropenia

Evans syndrome is a rare hematologic disorder characterized by the presence of simultaneous or sequential direct Coombs-positive autoimmune hemolytic anemia (AIHA), immune-mediated thrombocytopenia and/or immune neutropenia without any known underlying etiology. Spontaneous intracranial hemorrhage is a rare and life-threatening complication in patients with Evans syndrome and very few cases have been reported to date. We report a case of a thirty-two- year-old female with intracranial haemorrhage with underlying Evans syndrome who presented with the clinical manifestations of headache, vomiting and altered sensorium and succumbed to the fatal complication despite resuscitative measures. This also emphasizes the importance of early recognition of symptoms and immediate presentation to health care facilities for aggressive management of the patient.

scholarly journals A Female with Evans Syndrome Presented with Pancytopenia

2021 ◽  
Vol 10 (2) ◽  
pp. 114-117
Author(s):  
Md Rezaul Karim Chowdhury ◽  
Md Haroon Ur Rashid ◽  
Md Mahbub Hossain ◽  
Shafayet Hossain Riyan
Keyword(s):  
Oral Prednisolone ◽  
Low Grade ◽  
Evans Syndrome ◽  
First Line ◽  
Indirect Bilirubin ◽  
First Line Therapy ◽  
Line Therapy ◽  
Immune Neutropenia ◽  
Chronic Course

Evans syndrome is an uncommon haematological disorder characterised by autoimmune haemolytic anaemia (AIHA), immune thrombocytopenia (ITP) and/or immune neutropenia. It may occur in all ethnic groups, all ages and has no sex predilection. The direct antiglobulin test (DAT) is almost invariably positive. This condition generally runs a chronic course and is characterised by frequent exacerbations and remissions. Corticosteroids and/or intravenous immunoglobulin (IVIG) are the most commonly used first line therapy. Here we report a case of a female who presented with severe shortness of breath, palpitation and low grade fever and on examination she was found severely pale and mildly icteric. Her CBC and PBF showed pancytopenia. Indirect bilirubin and LDH were raised and direct Coomb’s test was positive. She was labeled as a case of Evans syndrome and responded to oral prednisolone. On subsequent follow-up her haematological profiles remained normal. J Enam Med Col 2020; 10(2): 114-117

scholarly journals Multicenter Study on Differential Human Neutrophil Antigen 2 Expression and Underlying Molecular Mechanisms

2020 ◽  
Vol 47 (5) ◽  
pp. 385-395
Author(s):  
Brigitte K. Flesch ◽  
Angelika Reil ◽  
Núria Nogués ◽  
Carme Canals ◽  
Peter Bugert ◽  
...  
Keyword(s):  
Expression Pattern ◽  
Human Neutrophil ◽  
Molecular Mechanisms ◽  
Normal Population ◽  
Regulatory Elements ◽  
Population Exposure ◽  
Nucleotide Polymorphisms ◽  
Single Nucleotide ◽  
Immune Neutropenia ◽  
The Impact

Background: The human neutrophil antigen 2 (HNA-2), which is expressed on CD177, is undetectable in 3–5% of the normal population. Exposure of these HNA-2null individuals to HNA-2-positive cells can cause immunization and pro­duction of HNA-2 antibodies, which can induce immune neutropenia and transfusion-related acute lung injury. In HNA-2-positive individuals, neutrophils are divided into a CD177pos. and a CD177neg. subpopulation. The molecular background of HNA-2 deficiency and the bimodal expression pattern, however, are not completely decoded. Study Design: An international collaboration was conducted on the genetic analysis of HNA-2-phenotyped blood samples, including HNA-2-deficient individuals, mothers, and the respective children with neonatal immune neutropenia and regular blood donors. Results: From a total of 54 HNA-2null individuals, 43 were homozygous for the CD177*787A>T substitution. Six carried the CD177*c.1291G>A single nucleotide polymorphism. All HNA-2-positive samples with >40% CD177pos. neutrophils carried the *787A wild-type allele, whereas a lower rate of CD177pos. neutrophils was preferentially associated with *c.787AT heterozygosity. Interestingly, only the *c.787A allele sequence was detected in complementary DNA (cDNA) sequence analysis carried out on all *c.787AT heterozygous individuals. However, cDNA analysis after sorting of CD177pos. and CD177neg. neutrophil subsets from HNA-2-positive individuals showed identical sequences, which makes regulatory elements within the promoter unlikely to affect CD177 gene transcription in different CD177 neutrophil subsets. Conclusion: This comprehensive study clearly demonstrates the impact of single nucleotide polymorphisms on the expression of HNA-2 on the neutrophil surface but challenges the hypothesis of regulatory epigenetic effects being implicated in the bimodal CD177 expression pattern.

Flecanide-Induced Immune Neutropenia

1987 ◽  
Vol 147 (2) ◽  
pp. 383 ◽  
Author(s):  
Wolfram E. Samlowski
Keyword(s):  
Immune Neutropenia

A Novel Method Using Extracted Human Neutrophil Antigens From HNA Gene-Transfected Cell Lines for Detection of Antibodies Against Human Neutrophil Antigens

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3272-3272
Author(s):  
Rie Onodera ◽  
Kazuhiro Nakamura ◽  
Kikuyo Taniguchi ◽  
Emi Kurita ◽  
Asako Hiraoka ◽  
...  
Keyword(s):  
Cell Lines ◽  
Positive Reaction ◽  
Human Neutrophil ◽  
Conflicts Of Interest ◽  
Age Distribution ◽  
Easy Method ◽  
Novel Method ◽  
Immune Neutropenia ◽  
Relationship Of ◽  
Normal Controls

Abstract Abstract 3272 Antibodies against Human Neutrophil Antigen (anti-HNA antibodies) have been implicated in immune neutropenia and transfusion-related acute lung injury. Those have been usually analyzed by Granulocyte indirect immunofluorescence test by flow-cytometry (GIFT-FCM), which requires fresh blood for panel neutrophils that cannot be preserved more than a few hours. Furthermore, specificity for anti-HNA antibodies cannot be easily identified by GIFT-FCM because of several antigens on membrane of panel neutrophil. In this study, we have developed a novel method designated as KY-Luminex Method, which was modified from Monoclonal antibody-specific immobilization of granulocyte antigens. For KY-Luminex Method, extracted human neutrophil antigens (eHNAs) were established from gene-transfected cell lines (KY-1a, -1b, -1c, -2a, -4a, -4b, -5a, -5b) that express HNA-1a, -1b, -1c, -2a, -4a, -4b, -5a, -5b, respectively. These eHNAs were immobilized by monoclonal antibodies on microspheres (eHNA-microspheres). Sera were tested to the eHNA-microspheres in wells of a 96-well microplate, respectively. The reactivities of the sera were analyzed by Luminex100. From May 2010 to March 2012, sera were obtained from 101 patients with autoimmune neutropenia in children. The age distribution was from 2 months to 72 months. Sixty-three patients were females and 38 were males. As shown in Table, the sera from 74 patients showed positive reaction both in standard GIFT-FCM and KY-Luminex method. The sera from 11 patients showed positive in GIFT-FCM, and negative in KY-Luminex. These sera finally proved to have antibodies against antigens other than neutrophil such as anti-HLA antibody. The sera from 13 patients showed negative in GIFT-FCM, and positive in KY-Luminex. The sera from 3 patients showed negative both in GIFT-FCM and KY-Luminex. Sera with positive reaction against each panel neutrophil in GIFT-FCM have tendency to react against multiple antigens in KY-luminex method. Especially, majoryty of analyzed sera had antibodies against HNA-4a, 4b, 5a and 5b. No antibodies against each antigens were detected in sera from normal controls by KY-luminex method. These results indicate that KY-luminex method has superior to GIFT-FCM in both sensitivity and specificity of anti-HNA antibodies. Furthermore, eHNA-microspheres are stable at 4 centigrade for more than a half year, and many samples such as 96 samples could be analyzes at once in simple and easy method. Table. Relationship of reactivities of patient's sera between GIFT-FCM and KY-luminex method. Data represent the number in 101 patients GIFT-FCM KY-Luminex method positive negative positive 74 11 negative 13 3 Disclosures: No relevant conflicts of interest to declare.

scholarly journals Anti-TNF therapy induced immune neutropenia in Crohns disease- report of 2 cases and review of literature

2012 ◽  
Vol 6 (6) ◽  
pp. 713-716 ◽  
Author(s):  
Shaji Sebastian ◽  
Katherine Ashton ◽  
Yasmine Houston ◽  
Tina Mary Diggory ◽  
Philip Dore
Keyword(s):  
Review Of Literature ◽  
Crohns Disease ◽  
Immune Neutropenia

scholarly journals Is There a Role for Anti-Neutrophil Antibody Testing in Predicting Spontaneous Resolution of Neutropenia in Young Children

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2211-2211 ◽  
Author(s):  
Laurence A. Boxer ◽  
Audrey Anna Bolyard ◽  
Tracy M. Marrero ◽  
Emily L. Tran ◽  
Mary Ann Bonilla ◽  
...  
Keyword(s):  
Young Children ◽  
Antibody Test ◽  
Infectious Complications ◽  
T Test ◽  
Severe Neutropenia ◽  
Antibody Testing ◽  
Congenital Neutropenia ◽  
Exact Test ◽  
Severe Infections ◽  
Immune Neutropenia

Abstract Immune neutropenia is the most common referral to pediatric hematologists. Controversy exists as to whether anti-neutrophil antibody testing is necessary or helpful in establishing a diagnosis, predicting outcomes or establishing the necessity of treatment with G-CSF. It is also unclear if clinicians can separate immune neutropenia and hereditary/congenital neutropenia based on patients' clinical presentations, blood counts and anti-neutrophil antibody testing. We reviewed the records of 60 patients with the clinical diagnosis of autoimmune or idiopathic neutropenia, enrolled in the Severe Chronic Neutropenia International Registry (SCNIR) who had the onset of neutropenia before age 2 and who were tested for anti-neutrophil antibodies (positive or negative) to see if they resolved neutropenia by age 7 years. We identified 36 antibody positive and 24 antibody negative patients. Neutropenia resolved by age 7 in 27/36 (75%) antibody positive and in 15/24 (62.5%) antibody negative patients (p = 0.3910, Fisher's exact test). The median age at recovery for those with resolving neutropenia was 3.10 years for the antibody positive and 3.60 years for antibody negative patients (mean ages 3.39 and 3.52 years, respectively; p = 0.7614, unpaired t-test). All 60 patients had severe neutropenia (mean ANC 0.376 x 109/L ± 0.620, median 0.193 x 109/L) and history of recurrent fevers and infections, and were treated with G-CSF (mean dose antibody positive 3.12 ± 7.22 mcg/kg/day, mean dose antibody negative 2.57 ± 3.21 mcg/kg/day, p = 0.7311, unpaired t-test; median doses 1.42 and 1.53 mcg/kg/day, respectively). The ANC responses were quite similar, with antibody positive patients' mean ANC on treatment 3.36 x 109/L ± 1.48; antibody negative mean ANC on treatment 4.10 x 109/L ± 2.06 (p = 0.107, unpaired t-test; median ANCs 1.83 and 2.42 x 109/L, respectively). There were no apparent differences in demographic or hematological characteristics of the patients that resolved or failed to resolve. We also identified 8 children with ELANE mutations who had a positive test for anti-neutrophil antibodies. A positive anti-neutrophil antibody result is generally thought to predict a benign course and favor withholding treatment with G-CSF. Six of eight of these children did not receive G-CSF until after the development of severe infections, which included pneumonia, liver abscess, and cellulitis, including one who developed severe cellulitis with the loss of a lower extremity. Importantly, neutropenia did not resolve in any of the ELANE mutated patients. This retrospective review demonstrates that anti-neutrophil antibody testing in young children is of very limited or perhaps no value in predicting prognosis or response to G-CSF treatment. Furthermore, the results of the antibody testing may lead to withholding G-CSF therapy from children who are at risk of severe infectious complications. We believe it is prudent to treat young children with severe neutropenia who have recurrent fevers and infections independent of the anti-neutrophil antibody test results. We also advocate genetic testing to detect congenital neutropenia and predict outcomes in these patients. Disclosures Boxer: Amgen: Equity Ownership. Dale:Amgen: Consultancy, Honoraria, Research Funding.

scholarly journals Autoimmune neutropenia [see comments]

Blood ◽  
1993 ◽  
Vol 81 (8) ◽  
pp. 1984-1995 ◽  
Author(s):  
KA Shastri ◽  
GL Logue
Keyword(s):  
Growth Factors ◽  
Neutrophil Function ◽  
Precursor Cells ◽  
Autoimmune Neutropenia ◽  
Hematopoietic Growth Factors ◽  
New Developments ◽  
The Past ◽  
Clinical Syndromes ◽  
Chronic Idiopathic Neutropenia ◽  
Immune Neutropenia

There have been several new developments in the field of autoimmune neutropenia over the past decade. Neutropenia caused by antibodies directed against granulocyte precursor cells, the oligoclonal nature of antineutrophil antibodies, and the expanding knowledge of neutrophil antigens, particularly in relationship to autoantibodies, are exciting new areas of investigation. Knowledge has also been advanced in the effector mechanisms of neutrophil autoantibodies and the effect of autoantibodies on the neutrophil function. In addition, some clinical syndromes of immune neutropenia have been better defined over the past decade, such as autoimmune neutropenia of infancy and chronic idiopathic neutropenia in adults. The past decade also saw interesting developments in the treatment of immune neutropenia, particularly in the use of gammaglobulin preparations and more recently in the advent of hematopoietic growth factors. This review focuses on these newer aspects of autoimmune neutropenia.

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