Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Mar 21;14(3):e23368.
doi: 10.7759/cureus.23368. eCollection 2022 Mar.

Hemophagocytic Lymphohistiocytosis: A Rare Cause of Pyrexia of Unknown Origin

Affiliations
Case Reports

Hemophagocytic Lymphohistiocytosis: A Rare Cause of Pyrexia of Unknown Origin

Salman Khan et al. Cureus. .

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome due to excessive immune activation leading to hyperinflammation. It may be familial due to mutations in immune regulatory genes, especially genetic defects of lymphocyte toxicity. The sporadic cases are triggered by infections (mostly viral), malignancies, and autoimmune diseases. Herewith we report the case of a 20-year-old male with febrile illness who was ultimately diagnosed with HLH.

Keywords: cytopenias; fever; hemophagocytic lymphohistiocytosis (hlh); immune activation; inflammation.

PubMed Disclaimer

Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Bone marrow aspirate of the patient showing hemophagocytosis
Figure 2
Figure 2. CT scan image of the patient's abdomen showing enlarged spleen

References

    1. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Filipovich A, McClain K, Grom A. Biol Blood Marrow Transplant. 2010;16:82–89. - PubMed
    1. Clinical characteristics, prognostic factors, and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Otrock ZK, Eby CS. Am J Hematol. 2015;90:220–224. - PubMed
    1. Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Henter JI, Horne A, Aricó M, et al. Pediatr Blood Cancer. 2007;48:124–131. - PubMed
    1. Characteristic immune abnormalities in hemophagocytic lymphohistiocytosis. Egeler RM, Shapiro R, Loechelt B, Filipovich A. J Pediatr Hematol Oncol. 1996;18:340–345. - PubMed
    1. Inherited defects in lymphocyte cytotoxic activity. Pachlopnik Schmid J, Côte M, Ménager MM, et al. Immunol Rev. 2010;235:10–23. - PubMed

Publication types

LinkOut - more resources