SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN A CHILD WITH CONCOMITANT EBSTEIN BARR VIRUS AND SCRUB TYPHUS INFECTION – A RARE ENTITY

2021 ◽  
pp. 27-28
Author(s):  
Saheli Dasgupta ◽  
Saumen Meur ◽  
Monideepa Dutta
Keyword(s):  
Scrub Typhus ◽  
Pediatric Population ◽  
Laboratory Data ◽  
Maculopapular Rash ◽  
Distinct Region ◽  
Barr Virus ◽  
Ebstein Barr Virus ◽  
Life Threatening ◽  
Most Probable Explanation ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Scrub typhus is a life threatening zoonosis caused by Orientia tsutsugamushi organisms that are transmitted by the larvae of trombiculid mites. It is endemic to a geographically distinct region, the so called Tsutsugamushi triangle, which includes Japan, China and South Korea. The disease is more prevalent in southern and northern India.It is characterised by focal or disseminated vasculitis and perivasculitis involving the lungs, liver, spleen and central nervous system. We report our experience with pediatric scrub typhus at a hospital in eastern India with EBV positive and secondary HLH. An 8 year old boy with fever, maculopapular rash, hepatosplenomegaly and lymphadenopathy was admitted in our institution and diagnosed with Scrub typhus. Physical and laboratory data showed hepatosplenomegaly, bicytopenia, hyperferritinemia, and hypobrinogenemia. Secondary HLH was diagnosed and the child was managed with IVIG and steroids. In view of rash followed by lymphadenopathy and hepatosplenomegaly EBV serology was also sent as a cause of HLH. Surprisingly, EBV panel was also positive. We therefore concluded that the most probable explanation was EBV triggered HPS following scrub typhus infection. Another possible explanation is EBV can be reactivated in critically ill patients. To our knowledge this is the rst such case in the pediatric population reported till date.

Continuous Renal Replacement Therapy with oXiris® Membrane in Severe Ebstein-Barr Virus-Mediated Hemophagocytic Lymphohistiocytosis: A Case Report

2020 ◽  
pp. 1-4
Author(s):  
Karla N. Samman ◽  
Hussein Baalbaki ◽  
Josée Bouchard ◽  
Martin Albert
Keyword(s):  
Renal Replacement Therapy ◽  
Continuous Renal Replacement Therapy ◽  
Replacement Therapy ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Inflammatory Markers ◽  
Blood Purification ◽  
Cytokine Storm ◽  
Barr Virus ◽  
Ebstein Barr Virus ◽  
Life Threatening

Hemophagocytic lymphohistiocytosis (HLH), a life-threatening disease with uncontrolled immune activation and inflammatory reaction, often leads to a deadly cytokine storm. In severe Ebstein-Barr virus-triggered HLH receiving standard immunosuppression, continuous renal replacement therapy (CRRT) with oXiris<sup>®</sup> blood purification membrane resulted in a timely reduction of inflammatory markers and discontinuation of vasopressors. To our knowledge, this is the first report of successful use of the oXiris<sup>®</sup> membrane in HLH.

Cutaneous Adverse Events of Immune Checkpoint Inhibitors: A Summarized Overview

2019 ◽  
Vol 14 (1) ◽  
pp. 14-20 ◽  
Author(s):  
Kerasia-Maria Plachouri ◽  
Eleftheria Vryzaki ◽  
Sophia Georgiou
Keyword(s):  
Side Effects ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitors ◽  
Checkpoint Inhibitors ◽  
Skin Toxicity ◽  
Maculopapular Rash ◽  
Symptomatic Treatment ◽  
Stevens Johnson Syndrome ◽  
Life Threatening ◽  
Skin Side Effects

Background:The introduction of Immune Checkpoint Inhibitors in the recent years has resulted in high response rates and extended survival in patients with metastatic/advanced malignancies. Their mechanism of action is the indirect activation of cytotoxic T-cells through the blockade of inhibitory receptors of immunomodulatory pathways, such as cytotoxic T-lymphocyte-associated antigen-4 (CTLA-4), programmed cell death protein-1 (PD-1) and its ligand (PD-L1). Despite their impressive therapeutic results, they can also induce immune-related toxicity, affecting various organs, including the skin.Objective:To provide an updated summarized overview of the most common immune-mediated cutaneous side effects and their management.Method:English articles derived from the databases PubMed and SCOPUS and published between 2009 and 2018, were analyzed for this narrative review.Results:The most common adverse cutaneous reactions include maculopapular rash, lichenoid reactions, vitiligo and pruritus, with severity Grade 1 or 2. Less frequent but eventually life-threatening skin side effects, including Stevens-Johnson syndrome, Drug Reaction with Eosinophilia and Systemic Symptoms and Toxic Epidermal necrolysis, have also been reported.Conclusion:Basic knowledge of the Immune-Checkpoint-Inhibitors-induced skin toxicity is necessary in order to recognize these treatment-related complications. The most frequent skin side effects, such as maculopapular rash, vitiligo and pruritus, tend to subside under symptomatic treatment so that permanent discontinuation of therapy is not commonly necessary. In the case of life-threatening side effects, apart from the necessary symptomatic treatment, the immunotherapy should be permanently stopped. Information concerning the management of ICIs-mediated skin toxicity can be obtained from the literature as well as from the Summary of Product Characteristics of each agent.

Intraorbital Abscess: A Rare Cause of Orbit Compression

FACE ◽  
2021 ◽  
pp. 273250162110050
Author(s):  
Samuel Ruiz ◽  
Rizal Lim
Keyword(s):  
Pediatric Population ◽  
Rare Complication ◽  
Disease Process ◽  
Rare Condition ◽  
External Drainage ◽  
Direct Extension ◽  
Life Threatening ◽  
History Of ◽  
High Index Of Suspicion ◽  
Prompt Diagnosis

Introduction: Intraorbital abscess is a rare complication of rhinosinusitis that affects most commonly the pediatric population. It is thought to be caused by direct extension or venous spread of infections from contiguous sites and can lead to life-threatening complications, like permanent visual loss and cerebral abscesses. Objectives: Intraorbital abscess is a rare condition that requires prompt diagnosis and treatment to avoid serious complications. Our objectives are to provide an overview of this rare disease process and its management including our successful treatment experience. Case Description: We present a 2 case report of a 13-year-old pediatric male and a 66-year-old male with history of chronic sinusitis who presented with a right intraorbital abscess successfully treated with external drainage with decompression of the orbit. Conclusion: When intraorbital abscess is encountered, a high index of suspicion is needed to allow prompt and accurate diagnosis for this infrequent condition. Timely surgical drainage of the abscess is needed to prevent the development of fatal complications.

scholarly journals Modulation of Endosome Function, Vesicle Trafficking and Autophagy by Human Herpesviruses

Cells ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 542
Author(s):  
Eduardo I. Tognarelli ◽  
Antonia Reyes ◽  
Nicolás Corrales ◽  
Leandro J. Carreño ◽  
Susan M. Bueno ◽  
...  
Keyword(s):  
Membrane Trafficking ◽  
Viral Gene ◽  
Barr Virus ◽  
Cellular Processes ◽  
Antiviral Therapies ◽  
Host Determinants ◽  
Viral Particles ◽  
Life Threatening ◽  
Epstein Barr ◽  
Human Herpesviruses

Human herpesviruses are a ubiquitous family of viruses that infect individuals of all ages and are present at a high prevalence worldwide. Herpesviruses are responsible for a broad spectrum of diseases, ranging from skin and mucosal lesions to blindness and life-threatening encephalitis, and some of them, such as Kaposi’s sarcoma-associated herpesvirus (KSHV) and Epstein–Barr virus (EBV), are known to be oncogenic. Furthermore, recent studies suggest that some herpesviruses may be associated with developing neurodegenerative diseases. These viruses can establish lifelong infections in the host and remain in a latent state with periodic reactivations. To achieve infection and yield new infectious viral particles, these viruses require and interact with molecular host determinants for supporting their replication and spread. Important sets of cellular factors involved in the lifecycle of herpesviruses are those participating in intracellular membrane trafficking pathways, as well as autophagic-based organelle recycling processes. These cellular processes are required by these viruses for cell entry and exit steps. Here, we review and discuss recent findings related to how herpesviruses exploit vesicular trafficking and autophagy components by using both host and viral gene products to promote the import and export of infectious viral particles from and to the extracellular environment. Understanding how herpesviruses modulate autophagy, endolysosomal and secretory pathways, as well as other prominent trafficking vesicles within the cell, could enable the engineering of novel antiviral therapies to treat these viruses and counteract their negative health effects.

scholarly journals Death of unknown cause? Post-mortem diagnosis of fulminant course of an EBV-associated secondary hemophagocytic lymphohistiocytosis

2021 ◽  
Author(s):  
Josia Fauser ◽  
Stefan Köck ◽  
Eberhard Gunsilius ◽  
Andreas Chott ◽  
Andreas Peer ◽  
...  
Keyword(s):  
Immune Response ◽  
Case Report ◽  
Macrophage Activation ◽  
Post Mortem ◽  
Life Threatening ◽  
Appropriate Therapy ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up ◽  
Secondary Hemophagocytic Lymphohistiocytosis

SummaryHLH is a life-threatening disease, which is characterized by a dysregulated immune response with uncontrolled T cell and macrophage activation. The often fulminant course of the disease needs a fast diagnostic work-up to initiate as soon as possible the appropriate therapy. We present herein the case of a 71-year-old patient with rapidly progressive hyperinflammatory syndrome, which post mortem resulted in the diagnosis of EBV-associated HLH. With this case report, we intend to highlight the relevance of the HScore in the diagnosis of HLH, to create a greater awareness for EBV as a trigger of HLH, and to demonstrate the importance of treating EBV-associated HLH as early as possible.

Clinical profile and predictors of severity in paediatric scrub typhus with pulmonary involvement

2021 ◽  
pp. 004947552110125
Author(s):  
Dinesh Kumar Narayanasamy ◽  
Thirunavukkarasu Arun Babu ◽  
Prakash Mathiyalagen
Keyword(s):  
Logistic Regression ◽  
Regression Analysis ◽  
Interstitial Pneumonitis ◽  
Clinical Characteristics ◽  
Scrub Typhus ◽  
Radiographic Finding ◽  
Maculopapular Rash ◽  
Pulmonary Involvement ◽  
Clearance Time ◽  
Fever Clearance Time

Pulmonary involvement is common in children with scrub typhus. Our paper outlines the clinical characteristics of pulmonary involvement and analyses the predictors of its severity. All scrub typhus serology-positive (optical density >0.5) children with pulmonary symptoms were included. Of 506 serology-positive scrub typhus cases, 256 (50.5%) had pulmonary symptoms, of whom 50 (9.8%) were severe. These severe cases were compared with non-severe cases. Interstitial pneumonitis was the commonest chest radiographic finding. Logistic regression analysis identified ‘fever clearance time’ >48 h, facial puffiness, maculopapular rash and anaemia to be significantly associated with severe pulmonary involvement.

scholarly journals Recurrent Ebstein-Barr Virus–Associated Diffuse Large B-Cell Lymphoma in an Ascending Aorta Graft

Circulation ◽  
2013 ◽  
Vol 128 (13) ◽  
pp. 1481-1483 ◽  
Author(s):  
Crystal R. Bonnichsen ◽  
Joseph A. Dearani ◽  
Joseph J. Maleszewski ◽  
Joseph P. Colgan ◽  
Eric E. Williamson ◽  
...  
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Ascending Aorta ◽  
Barr Virus ◽  
Large B Cell Lymphoma ◽  
Ebstein Barr Virus ◽  
Large B Cell

MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN ASSOCIATED WITH COVID-19 IN THE 6-YEAR-OLD PATIENT WITH MANIFESTATION OF TYPE 1 DIABETES MELLITUS WITH DIABETIC KETOACIDOSIS

2021 ◽  
Vol 100 (2) ◽  
pp. 295-300
Author(s):  
Yu.V. Tikhonovich ◽  
◽  
A.Yu. Rtishchev ◽  
A.A. Glazyrina ◽  
D.Yu. Ovsyannikov ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type 1 Diabetes ◽  
Type 1 Diabetes Mellitus ◽  
Diabetic Ketoacidosis ◽  
Clinical Observation ◽  
Laboratory Data ◽  
Domestic Literature ◽  
Life Threatening ◽  
Inflammatory Syndrome

For the first time in the domestic literature, the article presents a clinical observation of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 in the 6-year-old patient with manifestation of type 1 diabetes mellitus (T1DM) in the form of diabetic ketoacidosis. Anamnestic, clinical and laboratory data are presented on the basis of which two life-threatening diseases was diagnosed, as well as tactics of therapy, which made it possible to achieve a positive result. This clinical observation is compared with observations of foreign colleagues. Possible pathogenetic mechanisms of MIS-C and T1DM comorbidity are discussed.

Reporting altered test results in hemolyzed samples: is the cure worse than the disease?

2017 ◽  
Vol 55 (8) ◽  
pp. 1112-1114 ◽  
Author(s):  
Giuseppe Lippi ◽  
Gianfranco Cervellin ◽  
Mario Plebani
Keyword(s):  
Laboratory Data ◽  
Turnaround Time ◽  
Reliable Data ◽  
Test Results ◽  
The Past ◽  
Laboratory Test Results ◽  
Performance Specifications ◽  
Life Threatening ◽  
The Many ◽  
Definition Of

AbstractThe management of laboratory data in unsuitable (hemolyzed) samples remains an almost unresolved dilemma. Whether or not laboratory test results obtained by measuring unsuitable specimens should be made available to the clinicians has been the matter of fierce debates over the past decades. Recently, an intriguing alternative to suppressing test results and recollecting the specimen has been put forward, entailing the definition and implementation of specific algorithms that would finally allow reporting a preanalytically altered laboratory value within a specific comment about its uncertainty of measurement. This approach carries some advantages, namely the timely communication of potentially life-threatening laboratory values, but also some drawbacks. These especially include the challenging definition of validated performance specifications for hemolyzed samples, the need to producing reliable data with the lowest possible uncertainty, the short turnaround time for repeating most laboratory tests, the risk that the comments may be overlooked in short-stay and frequently overcrowded units (e.g. the emergency department), as well as the many clinical advantages of a direct communication with the physician in charge of the patient. Despite the debate remains open, we continue supporting the suggestion that suppressing data in unsuitable (hemolyzed) samples and promptly notifying the clinicians about the need to recollect the samples remains the most (clinically and analytically) safe practice.

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