Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still's Disease

Bhupen Barman; Md Jamil; Pranjal Kalita; Biswajit Dey

doi:10.7759/cureus.18037

Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still's Disease

Cureus. 2021 Sep 17;13(9):e18037. doi: 10.7759/cureus.18037. eCollection 2021 Sep.

Authors

Bhupen Barman¹, Md Jamil¹, Pranjal Kalita², Biswajit Dey²

Affiliations

¹ Internal Medicine, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), Shillong, IND.
² Pathology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences (NEIGRIHMS), Shillong, IND.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare hematological condition resulting from dysregulation of the immune system. This unusual clinical syndrome is characterized by fever, cytopenia, liver dysfunction, increased ferritin level, and evidence of hemophagocytosis in the bone marrow. We report a case of a 21-year-old female who presented with recurrent high-grade fever, transient rash, and polyarthritis who was subsequently diagnosed with adult-onset Still's disease (AOSD) with secondary HLH. The patient improved with aggressive management. Our case highlights HLH as a life-threatening and underdiagnosed complication of AOSD.

Keywords: adult-onset still’s disease; arthritis; cell-mediated immunity; immunity; secondary hemophagocytic lymphohistiocytosis.

Publication types

Case Reports