Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis

Wasylyshyn, Anastasia; Maki, Gina; Linder, Kathleen A.; Herc, Erica

doi:10.1097/ipc.0000000000001087

Cited by 3 publications

(2 citation statements)

References 17 publications

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“…The mechanism of histoplasmosis-triggered HLH has not been fully explained. However, it is likely related to a T-cell-mediated immune response, macrophage activation, and cytokine storm to an underlying trigger [5,9]. Mortality with HLH-associated histoplasmosis has a fatality rate of 31% [5,10].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Disseminated Histoplasmosis With Hemophagocytic Lymphohistiocytosis Mimicking a Flare of Systemic Lupus Erythematosus in a Middle-Aged Man: A Case Report

Omo-Ogboi,

Shirai,

Ur Rehman

et al. 2023

Cureus

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Disseminated histoplasmosis is a progressive granulomatous disease caused by Histoplasma capsulatum, which is an intracellular dimorphic fungus endemic to the Ohio and Mississippi River valleys in the United States. It is usually thought to be due to the failure of the activation of the T-cell-mediated immune response. Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal condition, in which histiocytes and lymphocytes build up in and damage organs and other blood cells. We present a 37-year-old man with a past medical history of systemic lupus erythematosus (SLE) complicated by lupus nephritis on immunosuppressive therapy who presented to the emergency department with hypotension and was admitted for acute kidney injury. Prior to the presentation, he had persistent fever, myalgias, cough, mild shortness of breath, and back pain. Computed tomography (CT) chest shows "eggshell" calcification; microbiology evaluation of peripheral blood smear revealed intracellular organism, morphologically consistent with H. capsulatum; and urine histoplasmosis antigen test confirmed the diagnosis of histoplasmosis. HLH diagnosis was made clinically after "clinical and testing criteria" were evaluated. Despite further management, he developed coagulopathy and sepsis, which led to his death. At autopsy, we found organomegaly of the liver, spleen, and kidneys. Microscopically, these enlarged organs show old fibrotic granulomas and granulomatous inflammation with suspected fungal organisms. Gomori's methenamine silver special stain confirmed these fungal organisms to be consistent with Histoplasma species (3-5 micron budding yeasts). This case highlights that physicians should be aware of the diagnostic challenge that disseminated histoplasmosis with HLH could pose in a patient with SLE, especially in patients on immunosuppression. Failure to recognize the infection promptly could lead to grievous complications and possibly death.

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Section: Discussionmentioning

confidence: 99%

A Rare Case of Disseminated Histoplasmosis With Hemophagocytic Lymphohistiocytosis Mimicking a Flare of Systemic Lupus Erythematosus in a Middle-Aged Man: A Case Report

Omo-Ogboi,

Shirai,

Ur Rehman

et al. 2023

Cureus

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show abstract

“…The mechanism of histoplasmosis triggered HLH has not been fully elucidated. However, it is likely related to a T-cell mediated immune response, macrophage activation and cytokine storm [3] , [16] . Mortality with HLH-associated histoplasmosis has a fatality rate of 31 % [17] .…”

Section: Discussionmentioning

confidence: 99%

Disseminated histoplasmosis and hemophagocytic lymphohistiocytosis in a patient receiving TNF-alpha inhibitor therapy

Zhong

Ordaya

Fernandez

et al. 2022

IDCases

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Multiple drugs

2022

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Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis

Abstract: Hemophagocytic lymphohistiocytosis is a syndrome of immune dysregulation that can lead to an overwhelming inflammatory state. In this case series, we describe 3 cases in which disseminated Histoplasma capsulatum infection caused hemophagocytic lymphohistiocytosis.

Cited by 3 publications

References 17 publications

A Rare Case of Disseminated Histoplasmosis With Hemophagocytic Lymphohistiocytosis Mimicking a Flare of Systemic Lupus Erythematosus in a Middle-Aged Man: A Case Report

A Rare Case of Disseminated Histoplasmosis With Hemophagocytic Lymphohistiocytosis Mimicking a Flare of Systemic Lupus Erythematosus in a Middle-Aged Man: A Case Report

Disseminated histoplasmosis and hemophagocytic lymphohistiocytosis in a patient receiving TNF-alpha inhibitor therapy

Multiple drugs

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