2018
DOI: 10.1016/j.mmcr.2018.01.001
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Hemophagocytic lymphohistiocytosis (HLH) secondary to disseminated histoplasmosis in the setting of Acquired Immunodeficiency Syndrome (AIDS)

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive disease involving immune system over-activation leading to hemophagocytosis. HLH requires early diagnosis and prompt treatment initiation, especially in patients with Acquired Immunodeficiency Syndrome (AIDS). We present a case of a middle-aged male with AIDS and renal failure, who developed HLH secondary to disseminated histoplasmosis. Etoposide chemotherapy as recommended by the HLH 2004 Guidelines was deferred and treatment focused instead on… Show more

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Cited by 15 publications
(23 citation statements)
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“…Red cell phagocytosis by macrophages on bone marrow examination can determine a diagnosis of HIHS. A comprehensive definition and classification of hemophagocytic lymphohistiocytosis are now available, but little is known regarding HIHS and no specific treatment guidance is available [45,46]. Severe clinical manifestations mimicking septic shock have been reported in around 10% of HAH cases and are probably caused by HIHS [5].…”
Section: Clinical Findingsmentioning
confidence: 99%
“…Red cell phagocytosis by macrophages on bone marrow examination can determine a diagnosis of HIHS. A comprehensive definition and classification of hemophagocytic lymphohistiocytosis are now available, but little is known regarding HIHS and no specific treatment guidance is available [45,46]. Severe clinical manifestations mimicking septic shock have been reported in around 10% of HAH cases and are probably caused by HIHS [5].…”
Section: Clinical Findingsmentioning
confidence: 99%
“…Treatment with tumor necrosis factor (TNF) inhibitors such as infliximab can also lead to increased risk of opportunistic infections particularly granulomatous diseases such as histoplasmosis and tuberculosis. Symptoms of disseminated histoplasmosis usually include fever, fatigue, weight loss, hepatosplenomegaly, lymphadenopathy, skin lesions, and pancytopenia [ 4 , 6 8 ]. There is considerable overlap of these symptoms with primary HLH, and the infection can itself trigger HLH.…”
Section: Discussionmentioning
confidence: 99%
“…Disseminated histoplasmosis is a fulminant infection which needs long-term systemic antifungal therapy. Immunosuppressive therapy can potentially worsen the underlying infection especially since histoplasmosis is typically seen to affect immunosuppressed individuals such as those with HIV or taking chemotherapy [3][4][5]. In this case report, we would like to discuss diagnosis and management of HLH and the spectrum of presentation of disseminated histoplasmosis and its treatment.…”
Section: Introductionmentioning
confidence: 99%
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“…Our patient, as well as other individuals with rheumatologic disorders (on immunosuppressant therapies), tend to have an opportunistic infection as the main trigger mechanism. Antitumor necrosis factor therapy (medication commonly used in autoimmune diseases) inhibits granuloma formation and increases the risk of systemic infections such as histoplasmosis [7].…”
Section: Discussionmentioning
confidence: 99%