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331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder that can be either primary (genetic) or secondary (reactive) in etiology. Diagnosis can be elusive, especially in patients with HIV infection. METHODS: Medical records were reviewed for 5 patients with HIV infection and the diag...

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Autores principales: Bhatt, Isha, Khan, Zeb, Lam, Paul, Quale, John M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6809881/
http://dx.doi.org/10.1093/ofid/ofz360.404
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author Bhatt, Isha
Khan, Zeb
Lam, Paul
Quale, John M
author_facet Bhatt, Isha
Khan, Zeb
Lam, Paul
Quale, John M
author_sort Bhatt, Isha
collection PubMed
description BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder that can be either primary (genetic) or secondary (reactive) in etiology. Diagnosis can be elusive, especially in patients with HIV infection. METHODS: Medical records were reviewed for 5 patients with HIV infection and the diagnosis of HLH. Standard and alternative criteria were utilized to establish the diagnosis. RESULTS: Five patients with HIV infection had clinical criteria for the diagnosis of HLH. Ages ranged from 33–70 years and 4 were males. All five presented with fevers, cytopenias, and markedly elevated ferritin levels (table). All of the patients had CD4 levels of < 200 cells/µL. Evidence of hemophagocytosis was found on bone marrow examination in 3 patients. Inciting conditions included Pneumocystis jiroveci infection, EBV infection, lymphoma, and multiple myeloma. All patients received broad-spectrum antimicrobial as well as immunosuppressive therapy. Despite aggressive treatments, all patients died within one month of presentation. CONCLUSION: In patients with underlying HIV infection, HLH can be a difficult diagnosis to establish. Mortality rates can be high, even with prompt recognition and therapy. The finding of fever and cytopenia in a patient with HIV infection should prompt the clinician to determine a ferritin level. If markedly elevated, the diagnosis of HLH should be aggressively pursued. [Image: see text] DISCLOSURES: All authors: No reported disclosures.
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spelling pubmed-68098812019-10-28 331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination Bhatt, Isha Khan, Zeb Lam, Paul Quale, John M Open Forum Infect Dis Abstracts BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder that can be either primary (genetic) or secondary (reactive) in etiology. Diagnosis can be elusive, especially in patients with HIV infection. METHODS: Medical records were reviewed for 5 patients with HIV infection and the diagnosis of HLH. Standard and alternative criteria were utilized to establish the diagnosis. RESULTS: Five patients with HIV infection had clinical criteria for the diagnosis of HLH. Ages ranged from 33–70 years and 4 were males. All five presented with fevers, cytopenias, and markedly elevated ferritin levels (table). All of the patients had CD4 levels of < 200 cells/µL. Evidence of hemophagocytosis was found on bone marrow examination in 3 patients. Inciting conditions included Pneumocystis jiroveci infection, EBV infection, lymphoma, and multiple myeloma. All patients received broad-spectrum antimicrobial as well as immunosuppressive therapy. Despite aggressive treatments, all patients died within one month of presentation. CONCLUSION: In patients with underlying HIV infection, HLH can be a difficult diagnosis to establish. Mortality rates can be high, even with prompt recognition and therapy. The finding of fever and cytopenia in a patient with HIV infection should prompt the clinician to determine a ferritin level. If markedly elevated, the diagnosis of HLH should be aggressively pursued. [Image: see text] DISCLOSURES: All authors: No reported disclosures. Oxford University Press 2019-10-23 /pmc/articles/PMC6809881/ http://dx.doi.org/10.1093/ofid/ofz360.404 Text en © The Author(s) 2019. Published by Oxford University Press on behalf of Infectious Diseases Society of America. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Abstracts
Bhatt, Isha
Khan, Zeb
Lam, Paul
Quale, John M
331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination
title 331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination
title_full 331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination
title_fullStr 331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination
title_full_unstemmed 331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination
title_short 331. Five Cases of Hemophagocytic Lymphohistiocytosis in Patients with HIV: A Fulminant and Lethal Combination
title_sort 331. five cases of hemophagocytic lymphohistiocytosis in patients with hiv: a fulminant and lethal combination
topic Abstracts
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6809881/
http://dx.doi.org/10.1093/ofid/ofz360.404
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