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      Hemophagocytic lymphohistiocytosis secondary to histoplasmosis: A case report in a patient with AIDS and recent SARS-CoV-2 infection and minireview

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          Abstract

          Here, we describe the case of a naïve HIV late presenter female African patient with progressive disseminated histoplasmosis and a severe life-threatening clinical picture in a non-endemic area. She had not visited Africa in the past decade. She developed a reactive hemophagocytic lymphohistiocytosis and an acute psychiatric disorder. Histoplasmosis was diagnosed after two bone marrow biopsies. Therapy with liposomal amphotericin B resulted in rapid and progressive improvements in blood examinations and clinical conditions, including the disappearance of psychiatric disorders. The characteristics of our case were compared with those of all other cases of hemophagocytic syndrome secondary to histoplasmosis in HIV-positive patients reported in PubMed. In conclusion, clinicians outside endemic areas should evaluate histoplasmosis as a cause of severe clinical picture, especially in a patient with a travel history to an endemic area, even after many years, considering the possible reactivation of latent infection.

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          Most cited references52

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          Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome.

          Because it has no unique clinical, biologic, or histologic features, reactive hemophagocytic syndrome may be difficult to distinguish from other diseases such as severe sepsis or hematologic malignancies. This study was undertaken to develop and validate a diagnostic score for reactive hemophagocytic syndrome.
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            Recommendations for the management of hemophagocytic lymphohistiocytosis in adults

            Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome induced by aberrantly activated macrophages and cytotoxic T cells. The primary (genetic) form, caused by mutations affecting lymphocyte cytotoxicity and immune regulation, is most common in children, whereas the secondary (acquired) form is most frequent in adults. Secondary HLH is commonly triggered by infections or malignancies but may also be induced by autoinflammatory/autoimmune disorders, in which case it is called macrophage activation syndrome (MAS; or MAS-HLH). Most information on the diagnosis and treatment of HLH comes from the pediatric literature. Although helpful in some adult cases, this raises several challenges. For example, the HLH-2004 diagnostic criteria developed for children are commonly applied but are not validated for adults. Another challenge in HLH diagnosis is that patients may present with a phenotype indistinguishable from sepsis or multiple organ dysfunction syndrome. Treatment algorithms targeting hyperinflammation are frequently based on pediatric protocols, such as HLH-94 and HLH-2004, which may result in overtreatment and unnecessary toxicity in adults. Therefore, dose reductions, individualized tailoring of treatment duration, and an age-dependent modified diagnostic approach are to be considered. Here, we present expert opinions derived from an interdisciplinary working group on adult HLH, sponsored by the Histiocyte Society, to facilitate knowledge transfer between physicians caring for pediatric and adult patients with HLH, with the aim to improve the outcome for adult patients affected by HLH.
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              Epidemiology of endemic systemic fungal infections in Latin America.

              Although endemic mycoses are a frequent health problem in Latin American countries, clinical and epidemiological data remain scarce and fragmentary. These mycoses have a significant impact on public health, and early diagnosis and appropriate treatment remain important. The target population for endemic disease in Latin America is mostly represented by low-income rural workers with limited access to a public or private health system. Unfortunately, diagnostic tools are not widely available in medical centers in Latin America; consequently, by the time patients are diagnosed with fungal infection, many are already severely ill. Among immunocompromised patients, endemic mycoses usually behave as opportunistic infections causing disseminated rather than localized disease. This paper reviews the epidemiology of the most clinically significant endemic mycoses in Latin America: paracoccidioidomycosis, histoplasmosis, and coccidioidomycosis. The burdens of disease, typically affected populations, and clinical outcomes also are discussed.
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                Author and article information

                Contributors
                Journal
                Heliyon
                Heliyon
                Heliyon
                Elsevier
                2405-8440
                21 July 2023
                August 2023
                21 July 2023
                : 9
                : 8
                : e18537
                Affiliations
                [a ]Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties “G D'Alessandro,” University of Palermo, Palermo, Italy
                [b ]Infectious and Tropical Disease Unit and Sicilian Regional Reference Center for the Fight Against AIDS, AOU Policlinico “P. Giaccone”, 90127 Palermo, Italy
                [c ]Palermo Fast-Track City, Casa dei Diritti, Via Libertà 45, 90143 Palermo, Italy
                [d ]Microbiology and Virology Unit- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties “G D'Alessandro,” University of Palermo, Palermo, Italy
                [e ]Unit of Pathology, Universital Hospital Paolo Giaccone, Palermo (PA), viale del vespro 147, Palermo, Italy
                Author notes
                []Corresponding author. PhD Infectious and Tropical Diseases Unit, AOUP P. Giaccone, Via del Vespro, 129, 90127 Palermo, Italy. antonio.cascio03@ 123456unipa.it
                Article
                S2405-8440(23)05745-6 e18537
                10.1016/j.heliyon.2023.e18537
                10392081
                b1a8c40c-f156-469f-936a-a1fcd390b2b7
                © 2023 The Authors

                This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

                History
                : 1 March 2023
                : 14 July 2023
                : 20 July 2023
                Categories
                Case Report

                histoplasmosis,aids,hiv,hemophagocytic lymphohistiocytosis

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