Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymp...

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Main Authors: Ramona Vesna Untanu, Syed Akbar, Stephen Graziano, Neerja Vajpayee
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Infectious Diseases
Online Access:http://dx.doi.org/10.1155/2016/1358742
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author Ramona Vesna Untanu
Syed Akbar
Stephen Graziano
Neerja Vajpayee
author_facet Ramona Vesna Untanu
Syed Akbar
Stephen Graziano
Neerja Vajpayee
author_sort Ramona Vesna Untanu
collection DOAJ
description Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymphocytes lead to production of large amounts of cytokines, also called cytokine storm. Hematopoietic and lymphoid tissues are directly involved while other organs are damaged by circulating cytokines. Primary HLH is attributed to genetic defects of the immune system and secondary HLH is usually seen in adults secondary to malignancy, infection, or autoimmune diseases. Zoonotic diseases including fungal infections are an important cause of HLH. Secondary HLH can delay the recognition of the underlying zoonoses. We report the case of a 61-year-old female with history of rheumatoid arthritis with histoplasmosis associated hemophagocytic lymphohistiocytosis.
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issn 2090-6625
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publishDate 2016-01-01
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spelling doaj-art-b9f45cd6a4a248bb90110614030a8ca52025-08-20T02:19:19ZengWileyCase Reports in Infectious Diseases2090-66252090-66332016-01-01201610.1155/2016/13587421358742Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the LiteratureRamona Vesna Untanu0Syed Akbar1Stephen Graziano2Neerja Vajpayee3Department of Pathology, SUNY Upstate Medical University, Syracuse, NY 13210, USADepartment of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY 13210, USADepartment of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY 13210, USADepartment of Pathology, SUNY Upstate Medical University, Syracuse, NY 13210, USAHemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threating immune dysregulation syndrome characterized by persistent activation of the mononuclear phagocytic system leading to uncontrolled systemic hyperinflammatory response. The proliferation and activation of histiocytes and lymphocytes lead to production of large amounts of cytokines, also called cytokine storm. Hematopoietic and lymphoid tissues are directly involved while other organs are damaged by circulating cytokines. Primary HLH is attributed to genetic defects of the immune system and secondary HLH is usually seen in adults secondary to malignancy, infection, or autoimmune diseases. Zoonotic diseases including fungal infections are an important cause of HLH. Secondary HLH can delay the recognition of the underlying zoonoses. We report the case of a 61-year-old female with history of rheumatoid arthritis with histoplasmosis associated hemophagocytic lymphohistiocytosis.http://dx.doi.org/10.1155/2016/1358742
spellingShingle Ramona Vesna Untanu
Syed Akbar
Stephen Graziano
Neerja Vajpayee
Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature
Case Reports in Infectious Diseases
title Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature
title_full Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature
title_fullStr Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature
title_full_unstemmed Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature
title_short Histoplasmosis-Induced Hemophagocytic Lymphohistiocytosis in an Adult Patient: A Case Report and Review of the Literature
title_sort histoplasmosis induced hemophagocytic lymphohistiocytosis in an adult patient a case report and review of the literature
url http://dx.doi.org/10.1155/2016/1358742
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