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Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis

Hemophagocytic lymphohistiocytosis (HLH) due to an underlying rheumatologic condition is known as macrophage activation syndrome (MAS), a rare and serious complication that often has a delayed diagnosis. MAS can complicate any rheumatologic disease, although it is most prevalent in systemic juvenile...

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Autores principales: Kasparian, Saro, Anand, Kartik, Burns, Ethan, Chung, Betty, Pingali, Sai Ravi Kiran
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6695236/
https://www.ncbi.nlm.nih.gov/pubmed/31431835
http://dx.doi.org/10.7759/cureus.4929
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author Kasparian, Saro
Anand, Kartik
Burns, Ethan
Chung, Betty
Pingali, Sai Ravi Kiran
author_facet Kasparian, Saro
Anand, Kartik
Burns, Ethan
Chung, Betty
Pingali, Sai Ravi Kiran
author_sort Kasparian, Saro
collection PubMed
description Hemophagocytic lymphohistiocytosis (HLH) due to an underlying rheumatologic condition is known as macrophage activation syndrome (MAS), a rare and serious complication that often has a delayed diagnosis. MAS can complicate any rheumatologic disease, although it is most prevalent in systemic juvenile idiopathic arthritis. MAS occurring as a sequela of sarcoidosis is seldom reported. Herein, we present an uncommon case of MAS occurring secondary to suspected extrapulmonary sarcoidosis and the associated diagnostic challenges. A 53-year-old White female presented with a 20-month history of constitutional symptoms of an unclear etiology. Her extensive workup included equivocal bone marrow and liver biopsies, suggestive of occasional hemophagocytosis. On admission, she met criteria for HLH based on the HLH-94 diagnostic guidelines. A repeat liver biopsy was performed revealing non-necrotizing granulomas in the parenchyma. Given the concern for an extrapulmonary sarcoidosis, she was started on pulse-dose steroids with subsequent symptomatic resolution. Two years later, she remains in complete remission. As a systemic disease, sarcoidosis can manifest in any organ and present in a variety of ways. While HLH and MAS have numerous etiologies, sarcoidosis should be considered as a potential underlying diagnosis, and prompt treatment initiation with steroids may reduce morbidity and mortality.
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spelling pubmed-66952362019-08-20 Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis Kasparian, Saro Anand, Kartik Burns, Ethan Chung, Betty Pingali, Sai Ravi Kiran Cureus Oncology Hemophagocytic lymphohistiocytosis (HLH) due to an underlying rheumatologic condition is known as macrophage activation syndrome (MAS), a rare and serious complication that often has a delayed diagnosis. MAS can complicate any rheumatologic disease, although it is most prevalent in systemic juvenile idiopathic arthritis. MAS occurring as a sequela of sarcoidosis is seldom reported. Herein, we present an uncommon case of MAS occurring secondary to suspected extrapulmonary sarcoidosis and the associated diagnostic challenges. A 53-year-old White female presented with a 20-month history of constitutional symptoms of an unclear etiology. Her extensive workup included equivocal bone marrow and liver biopsies, suggestive of occasional hemophagocytosis. On admission, she met criteria for HLH based on the HLH-94 diagnostic guidelines. A repeat liver biopsy was performed revealing non-necrotizing granulomas in the parenchyma. Given the concern for an extrapulmonary sarcoidosis, she was started on pulse-dose steroids with subsequent symptomatic resolution. Two years later, she remains in complete remission. As a systemic disease, sarcoidosis can manifest in any organ and present in a variety of ways. While HLH and MAS have numerous etiologies, sarcoidosis should be considered as a potential underlying diagnosis, and prompt treatment initiation with steroids may reduce morbidity and mortality. Cureus 2019-06-18 /pmc/articles/PMC6695236/ /pubmed/31431835 http://dx.doi.org/10.7759/cureus.4929 Text en Copyright © 2019, Kasparian et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Oncology
Kasparian, Saro
Anand, Kartik
Burns, Ethan
Chung, Betty
Pingali, Sai Ravi Kiran
Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis
title Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis
title_full Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis
title_fullStr Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis
title_full_unstemmed Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis
title_short Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis
title_sort macrophage activation syndrome secondary to underlying sarcoidosis
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6695236/
https://www.ncbi.nlm.nih.gov/pubmed/31431835
http://dx.doi.org/10.7759/cureus.4929
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