scholarly journals Identification of Antibodies Against Neutrophil Surface Antigens in Two Iranian Patients with Autoimmune Neutropenia

2021 ◽  
Author(s):  
Behnaz Esmaeili ◽  
Behnaz Bayat ◽  
Mohamad Reza Fazlollahi ◽  
Leila Moradi ◽  
Arash Kalantari ◽  
...  
Keyword(s):  
Surface Antigens ◽  
Final Diagnosis ◽  
Screening Tests ◽  
Autoimmune Neutropenia ◽  
Immunofluorescence Test ◽  
Immune Mediated ◽  
Positive Reactivity ◽  
Mediterranean Fever ◽  
Iranian Patients ◽  
Neutrophil Surface

Autoimmune neutropenia is a type of immune-mediated neutropenia, caused by antibody-induced neutrophil destruction. Here, we report two cases (a 3-year-old boy and a 9-year-old girl) with suspected autoimmune neutropenia. The presence of neutrophil antibodies in the sera of these two patients was investigated; using standard neutrophil antibody screening tests such as granulocyte immunofluorescence test (GIFT), granulocyte agglutination test (GAT), and lymphocyte immunofluorescence test (LIFT). A positive reactivity with two-panel cells was found in GIFT. No reactivities with panel cells were observed in GAT and LIFT. To the best of our knowledge, this is the first report for detecting the neutrophil reactive antibodies; using genotyped neutrophils in patients with autoimmune neutropenia in Iran. The final diagnosis of our patients was primary autoimmune neutropenia for the boy and autoimmune neutropenia associated with familial Mediterranean fever for the girl.

scholarly journals Oral Verruciform Xanthoma within Lichen Planus: A Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Vasileios I. Theofilou ◽  
Alexandra Sklavounou ◽  
Prokopios P. Argyris ◽  
Evanthia Chrysomali
Keyword(s):  
Case Report ◽  
Lichen Planus ◽  
Oral Lichen Planus ◽  
Cell Damage ◽  
Final Diagnosis ◽  
Verruciform Xanthoma ◽  
Immune Mediated ◽  
Wide Range ◽  
Oral Lichen

Background. Verruciform xanthoma is an uncommon benign tumor, which exhibits a wide range of clinical patterns. The occurrence of the lesion in patients with immune-mediated mucocutaneous diseases may suggest a role of localized epithelial cell damage and chronic inflammation in its pathogenesis. Case Report. A case of verruciform xanthoma on the tongue of a 56-year-old female with oral lichen planus is reported. An asymptomatic pink-white lesion with a granular surface was observed in the left lateral lingual border, which was closely associated with a white plaque and striae. An incisional biopsy was performed, and histologically, epithelial projections in a verrucous pattern were observed. In the subepithelial connective tissue, aggregates of foamy cells that exhibited immunoreactivity for CD68 were noted. The final diagnosis was verruciform xanthoma. The mucosa adjacent to the lesion demonstrated histopathological features consistent with lichen planus. Conclusions. A total of twelve cases of oral verruciform xanthomas in patients with oral lichen planus including the present case have been reported in the literature. The clinician should be aware that verruciform xanthoma may mimic malignancy, and therefore, biopsy is required for definitive diagnosis to be established, especially when this tumor develops within conditions that show potential for malignant transformation.

Understanding the “philosophy” of laboratory hemostasis

Diagnosis ◽  
2019 ◽  
Vol 6 (3) ◽  
pp. 223-226 ◽  
Author(s):  
Giuseppe Lippi ◽  
Dorothy Adcock ◽  
Emmanuel J. Favaloro
Keyword(s):  
Basic Concept ◽  
Clinical Medicine ◽  
Final Diagnosis ◽  
Laboratory Medicine ◽  
Diagnostic Reasoning ◽  
Screening Tests ◽  
Laboratory Diagnostics ◽  
Single Test ◽  
Biological Factors ◽  
Multiple Components

Abstract Unlike many other areas of laboratory medicine, laboratory hemostasis has some peculiarities, which makes it one of the most complex diagnostic domains in clinical medicine. The inherent complexity of the hemostasis process, the components of which have not yet been thoroughly unravelled, is mirrored by a large number of hemostasis disturbances, which can involve single or multiple components. Although laboratory diagnostics represents an unavoidable part of the diagnostic reasoning in patients with bleeding or thrombotic disorders, the basic concept beneath the assumption that in many human pathologies, one single test may be sufficient for the diagnosis, does not hold true in hemostasis. There are in fact many aspects that would lead us to conclude that laboratory hemostasis can be considered a very challenging arena for many clinicians and perhaps also for some laboratory professionals. The most challenging aspects typically include the following concepts; that hemostasis is an intricate and multifaceted process, that more than one test is typically needed to achieve a final diagnosis, that results of screening tests depend on many biological factors and do not allow making a final diagnosis, that harmonization of techniques is still an unmet target, and that the calculations used vary widely among laboratories. This article is hence aimed at discussing many of these aspects, with the hope of presenting a useful contribution to better understand the “philosophy” of laboratory hemostasis.

scholarly journals The use and limitation of labeled staphylococcal protein A for study of antineutrophil antibodies

Blood ◽  
1979 ◽  
Vol 54 (6) ◽  
pp. 1330-1337 ◽  
Author(s):  
JA McCallister ◽  
LA Boxer ◽  
RL Baehner
Keyword(s):  
Polymorphonuclear Leukocytes ◽  
Protein A ◽  
Surface Antigens ◽  
Staphylococcal Protein A ◽  
Lateral Movement ◽  
Autoimmune Neutropenia ◽  
Subsequent Removal ◽  
Staphylococcal Protein ◽  
Functional Consequences

Abstract Antineutrophil antibodies can be detected following their attachment to neutrophils by employing labeled staphylococcal protein A (SPA). Radiolabeled SPA provides a sensitive means for identifying the presence of IgG restricted to subclasses IgG1, IgG2, and IgG4 that will specifically bind to neutrophils and that are found in the serum of patients with isoimmune and autoimmune neutropenia. However, SPA bound to the Fc region of IgG does not interfere with the attachment of IgG to the Fc domain of the neutrophil. Fluorescein-labeled SPA, in turn is useful in monitoring the functional consequences of antibody attachment to the surface of polymorphonuclear leukocytes (PMN). Both heterologous and isoimmune antisera induced lateral movement of surface antigens into polar-capped pseudopodia. The formation of such pseudopodia may facilitate leukoagglutination and the subsequent removal of sensitized cells from the circulation.

scholarly journals Utility of Plasmapheresis in Autoimmune-Mediated Encephalopathy in Children: Potentials and Challenges

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Abdulhafeez M. Khair
Keyword(s):  
Intensive Care ◽  
Current Knowledge ◽  
Pediatric Population ◽  
Final Diagnosis ◽  
First Line ◽  
Clinical Value ◽  
First Line Therapy ◽  
Line Therapy ◽  
Immune Mediated ◽  
Early Use

Autoimmune-mediated encephalopathy in children continues to constitute a diagnostic and therapeutic challenge in pediatric population. Utility and usefulness in this clinical setting of plasmapheresis have seldom been evaluated in current pediatric literature. Children with immune-mediated encephalopathies represent a uniquely different group among patients presenting to intensive care units or neurological services worldwide. Arriving at a final diagnosis is not an easy task for treating physicians. It is very crucial to consider early use of first-line immunotherapy modalities, save those children’s lives and improve outcomes. Plasmapheresis is an emerging, potentially beneficial first-line therapy in such patients. However, indications, value, logistics, and procedural difficulties are often faced. This study is mainly meant to review the current knowledge in regard to the clinical value of plasmapheresis in children with immune-mediated encephalopathy.

scholarly journals Imaging and CSF analyses effectively distinguish CJD from its mimics

2017 ◽  
Vol 89 (5) ◽  
pp. 461-466 ◽  
Author(s):  
Peter Rudge ◽  
Harpreet Hyare ◽  
Alison Green ◽  
John Collinge ◽  
Simon Mead
Keyword(s):  
Lewy Bodies ◽  
Protein Detection ◽  
Clinical History ◽  
Final Diagnosis ◽  
Clinical Findings ◽  
Diffusion Weighted Mri ◽  
Diffusion Weighted ◽  
Immune Mediated ◽  
Jakob Disease ◽  
The Uk

ObjectiveTo review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis.MethodsReview the clinical findings and investigations in 214 patients enrolled into the UK National Prion Monitoring Cohort Study between October 2008 and November 2015 who had postmortem confirmed sCJD and compare these features with 50 patients referred over the same period who had an alternative final diagnosis (CJD mimics).ResultsPatients with an alternative diagnosis and those with sCJD were of similar age, sex and frequency of dementia but CJD mimics had a longer clinical history. Myoclonus, rigidity and hallucinations were more frequent in patients with sCJD but these features were not helpful in classifying individual patients. Alzheimer’s disease, dementia with Lewy bodies and genetic neurodegenerative disorders were alternative diagnoses in more than half of the CJD mimic cases, and 10% had an immune-mediated encephalopathy; lymphoma, hepatic encephalopathy and progressive multifocal leukoencephalopathy were seen more than once. Diffusion-weighted MRI was the most useful readily available test to classify cases correctly (92% CJD, 2% CJD mimics). The CSF cell count, 14-3-3 protein detection and S100B were of limited value. A positive CSF RT-QuIC test, introduced during the course of the study, was found in 89% of tested CJD cases and 0% CJD mimics.ConclusionThe combination of diffusion-weighted MRI analysis and CSF RT-QuIC allowed a perfect classification of sCJD versus its mimics in this study.

scholarly journals Acute cerebellar ataxia: differential diagnosis and clinical approach

2019 ◽  
Vol 77 (3) ◽  
pp. 184-193 ◽  
Author(s):  
José Luiz Pedroso ◽  
Thiago Cardoso Vale ◽  
Pedro Braga-Neto ◽  
Lívia Almeida Dutra ◽  
Marcondes Cavalcante França Jr ◽  
...  
Keyword(s):  
Cerebellar Ataxia ◽  
Healthy Subjects ◽  
Metabolic Diseases ◽  
Screening Tests ◽  
Common Finding ◽  
Clinical Approach ◽  
Initial Screening ◽  
Immune Mediated ◽  
Acute Cerebellar Ataxia ◽  
Cerebellar Lesions

ABSTRACT Cerebellar ataxia is a common finding in neurological practice and has a wide variety of causes, ranging from the chronic and slowly-progressive cerebellar degenerations to the acute cerebellar lesions due to infarction, edema and hemorrhage, configuring a true neurological emergency. Acute cerebellar ataxia is a syndrome that occurs in less than 72 hours, in previously healthy subjects. Acute ataxia usually results in hospitalization and extensive laboratory investigation. Clinicians are often faced with decisions on the extent and timing of the initial screening tests, particularly to detect treatable causes. The main group of diseases that may cause acute ataxias discussed in this article are: stroke, infectious, toxic, immune-mediated, paraneoplastic, vitamin deficiency, structural lesions and metabolic diseases. This review focuses on the etiologic and diagnostic considerations for acute ataxia.

Adult encephalitis surveillance: experiences from an Australian prospective sentinel site study

2015 ◽  
Vol 143 (15) ◽  
pp. 3300-3307 ◽  
Author(s):  
K. EASTWOOD ◽  
B. J. PATERSON ◽  
C. LEVI ◽  
R. GIVNEY ◽  
M. LOEWENTHAL ◽  
...  
Keyword(s):  
Hospital Admissions ◽  
Diagnostic Algorithm ◽  
Final Diagnosis ◽  
Case Definition ◽  
Sentinel Site ◽  
Emerging Pathogens ◽  
Immune Mediated ◽  
Data Source ◽  
Simplex Virus ◽  
Standardized Diagnostic

SUMMARYFew countries routinely collect comprehensive encephalitis data, yet understanding the epidemiology of this condition has value for clinical management, detecting novel and emerging pathogens, and guiding timely public health interventions. When this study was conducted there was no standardized diagnostic algorithm to aid identification of encephalitis or systematic surveillance for adult encephalitis. In July 2012 we tested three pragmatic surveillance options aimed at identifying possible adult encephalitis cases admitted to a major Australian hospital: hospital admissions searches, clinician notifications and laboratory test alerts (CSF herpes simplex virus requests). Eligible cases underwent structured laboratory investigation and a specialist panel arbitrated on the final diagnosis. One hundred and thirteen patients were initially recruited into the 10-month study; 20/113 (18%) met the study case definition, seven were diagnosed with infectious or immune-mediated encephalitis and the remainder were assigned alternative diagnoses. The laboratory alert identified 90% (102/113) of recruited cases including six of the seven cases of confirmed encephalitis suggesting that this may be a practical data source for case ascertainment. The application of a standardized diagnostic algorithm and specialist review by an expert clinical panel aided diagnosis of patients with encephalitis.

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