Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report

BackgroundAutoimmune neutropenia (AIN) is divided into primary and secondary forms. The former is more prevalent in children and is usually a self-limiting disease. Secondary AIN is more common in adults and often occurs in the setting of another autoimmune disorder or secondary to infections, malig...

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Main Authors: Kobi Faierstein, Noya Shilo, Asaf Levartovsky, Roy Raphael, Amir Givon, Nancy Agmon-Levin, Haim Mayan
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-05-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2022.880016/full
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author Kobi Faierstein
Kobi Faierstein
Noya Shilo
Noya Shilo
Asaf Levartovsky
Asaf Levartovsky
Roy Raphael
Roy Raphael
Amir Givon
Amir Givon
Nancy Agmon-Levin
Nancy Agmon-Levin
Haim Mayan
Haim Mayan
author_facet Kobi Faierstein
Kobi Faierstein
Noya Shilo
Noya Shilo
Asaf Levartovsky
Asaf Levartovsky
Roy Raphael
Roy Raphael
Amir Givon
Amir Givon
Nancy Agmon-Levin
Nancy Agmon-Levin
Haim Mayan
Haim Mayan
author_sort Kobi Faierstein
collection DOAJ
description BackgroundAutoimmune neutropenia (AIN) is divided into primary and secondary forms. The former is more prevalent in children and is usually a self-limiting disease. Secondary AIN is more common in adults and often occurs in the setting of another autoimmune disorder or secondary to infections, malignancies or medications. Several viral and bacterial pathogens were described to trigger AIN. Here we report a case of AIN in an adult woman associated with human herpesvirus-6 (HHV-6) infection.Case PresentationWe report a case of AIN in an adult woman associated with HHV-6 infection. The patient presented to the emergency department with fever and painful genital ulcers. Upon arrival, her laboratory workup demonstrated severe neutropenia and elevated inflammatory markers. She was hospitalized and underwent a thorough infectious, hematological, autoimmune and inflammatory workup. Malignancy was also excluded using an advanced whole body radiological scan. Serological tests confirmed the presence of both acute and chronic types of HHV-6 antibodies, at very high titers. Polymerase chain reaction demonstrated a numerous copies of the virus in the patient’s blood. Specific immunofluorescence test confirmed the diagnosis of autoimmune neutropenia.ConclusionSecondary AIN is a rare disease that may affect all range of ages. The adult type is a challenging disorder that has different etiologies and may be triggered by a variable infectious pathogen. The finding of HHV-6 as a possible culprit pathogen may warrant physicians into widening the evaluation and include HHV-6 in the analysis.
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spelling doaj.art-80de05d0e48a4a58bd34bed69ab3cf5b2022-12-22T02:54:31ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-05-011310.3389/fimmu.2022.880016880016Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case ReportKobi Faierstein0Kobi Faierstein1Noya Shilo2Noya Shilo3Asaf Levartovsky4Asaf Levartovsky5Roy Raphael6Roy Raphael7Amir Givon8Amir Givon9Nancy Agmon-Levin10Nancy Agmon-Levin11Haim Mayan12Haim Mayan13Department of Medicine E, The Chaim Sheba Medical Center, Ramat-Gan, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelDepartment of Medicine E, The Chaim Sheba Medical Center, Ramat-Gan, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelDepartment of Medicine E, The Chaim Sheba Medical Center, Ramat-Gan, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelDepartment of Medicine E, The Chaim Sheba Medical Center, Ramat-Gan, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelDepartment of Medicine E, The Chaim Sheba Medical Center, Ramat-Gan, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelThe Clinical Immunology, Angioedema and Allergy Unit, Center for Autoimmune Diseases, Sheba Medical Center, Ramat-Gan, IsraelDepartment of Medicine E, The Chaim Sheba Medical Center, Ramat-Gan, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelBackgroundAutoimmune neutropenia (AIN) is divided into primary and secondary forms. The former is more prevalent in children and is usually a self-limiting disease. Secondary AIN is more common in adults and often occurs in the setting of another autoimmune disorder or secondary to infections, malignancies or medications. Several viral and bacterial pathogens were described to trigger AIN. Here we report a case of AIN in an adult woman associated with human herpesvirus-6 (HHV-6) infection.Case PresentationWe report a case of AIN in an adult woman associated with HHV-6 infection. The patient presented to the emergency department with fever and painful genital ulcers. Upon arrival, her laboratory workup demonstrated severe neutropenia and elevated inflammatory markers. She was hospitalized and underwent a thorough infectious, hematological, autoimmune and inflammatory workup. Malignancy was also excluded using an advanced whole body radiological scan. Serological tests confirmed the presence of both acute and chronic types of HHV-6 antibodies, at very high titers. Polymerase chain reaction demonstrated a numerous copies of the virus in the patient’s blood. Specific immunofluorescence test confirmed the diagnosis of autoimmune neutropenia.ConclusionSecondary AIN is a rare disease that may affect all range of ages. The adult type is a challenging disorder that has different etiologies and may be triggered by a variable infectious pathogen. The finding of HHV-6 as a possible culprit pathogen may warrant physicians into widening the evaluation and include HHV-6 in the analysis.https://www.frontiersin.org/articles/10.3389/fimmu.2022.880016/fullsecondary autoimmune neutropenia (AIN)human herpesvirus 6 (HHV-6)granulocyte immunofluorescence test (GIFT)agranulocitosisneutropenic fever
spellingShingle Kobi Faierstein
Kobi Faierstein
Noya Shilo
Noya Shilo
Asaf Levartovsky
Asaf Levartovsky
Roy Raphael
Roy Raphael
Amir Givon
Amir Givon
Nancy Agmon-Levin
Nancy Agmon-Levin
Haim Mayan
Haim Mayan
Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report
Frontiers in Immunology
secondary autoimmune neutropenia (AIN)
human herpesvirus 6 (HHV-6)
granulocyte immunofluorescence test (GIFT)
agranulocitosis
neutropenic fever
title Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report
title_full Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report
title_fullStr Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report
title_full_unstemmed Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report
title_short Autoimmune Neutropenia Associated With HHV-6 Virus Infection: A Case Report
title_sort autoimmune neutropenia associated with hhv 6 virus infection a case report
topic secondary autoimmune neutropenia (AIN)
human herpesvirus 6 (HHV-6)
granulocyte immunofluorescence test (GIFT)
agranulocitosis
neutropenic fever
url https://www.frontiersin.org/articles/10.3389/fimmu.2022.880016/full
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