Thumbnail
Access Restriction
Open

Author Casault, Colin ♦ Posadas-Calleja, Juan G.
Editor Doganay, Mehmet
Source Hindawi
Content type Text
Publisher Hindawi
File Format PDF
Copyright Year ©2019
Language English
Abstract Background. We describe a case of secondary Hemophagocytic Lymphohistiocytosis (HLH) from autoimmune hepatitis mimicking severe sepsis in a man admitted to the intensive care unit. Case Presentation. A 34-year-old Pakistani male with a prior history of biopsy-proven autoimmune hepatitis presented to a regional hospital with severe fever, cytopenias, hyperferritinemia, hypertriglyceridemia, splenomegaly, and a bone marrow biopsy showing hemophagocytosis. After ruling out mimicking conditions, a diagnosis of HLH was made using the HLH-2004 diagnostic criteria. He was treated with dexamethasone and etoposide, without bone marrow transplantation (BMT) due to poor functional status. At one-year after follow-up, he had returned to his baseline functional status without recurrence. Conclusion. We describe a rare case of secondary HLH in the setting of autoimmune hepatitis. Broadly, this case report educates clinicians to consider this potentially missed diagnosis. This case also informs clinicians that treatment of secondary HLH with BMT may not be necessary for the management of secondary HLH due to autoimmune hepatitis. Finally, it provides a detailed description of the natural history of a single patient with secondary HLH due to autoimmune hepatitis.
ISSN 20906420
Learning Resource Type Report
Publisher Date 2019-02-04
Rights License This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
e-ISSN 20906439
Journal Case Reports in Critical Care
Volume Number 2019
Page Count 4


Open content in new tab

   Open content in new tab