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
. 2025 Jun 30;17(4):33.
doi: 10.3390/hematolrep17040033.

Secondary Hemophagocytic Lymphocytosis in Inflammatory Bowel Disease

Affiliations
Case Reports

Secondary Hemophagocytic Lymphocytosis in Inflammatory Bowel Disease

Jacob Boccucci et al. Hematol Rep. .

Abstract

Background and Clinical Significance: Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening condition that can go underdiagnosed due to overlapping features with severe infections. While the use of thiopurine in inflammatory bowel disease (IBD) has been associated with HLH, the majority of these patients will have a concurrent Epstein-Barr virus (EBV) infection. Case Presentation: This report presents a case of HLH in a patient previously treated with aza-thioprine for IBD without concurrent viral infection.

Keywords: hemophagocytic lymphocytosis; inflammatory bowel disease; pancytopenia.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
(a) Slide showing histiocytes displaying round contour with cytoplasmic projections which, combined with the other HLH-2004 criteria, give a diagnosis of HLH; (b) Activated histiocytes with expended cytoplasm and engulfed RBCs, lymphocytes, and PMN.

Similar articles

References

    1. Abdelhay A., Mahmoud A.A., Al Ali O., Hashem A., Orakzai A., Jamshed S. Epidemiology, characteristics, and outcomes of adult haemophagocytic lymphohistiocytosis in the USA, 2006–2019: A national, retrospective cohort study. EClinicalMedicine. 2023;62:102143. doi: 10.1016/j.eclinm.2023.102143. - DOI - PMC - PubMed
    1. Ramos-Casals M., Brito-Zeron P., Lopez-Guillermo A., Khamashta M.A., Bosch X. Adult haemophagocytic syndrome. Lancet. 2014;383:1503–1516. doi: 10.1016/S0140-6736(13)61048-X. - DOI - PubMed
    1. Janka G.E., Lehmberg K. Hemophagocytic lymphohistiocytosis: Pathogenesis and treatment. Hematol. Am. Soc. Hematol. Educ. Program. 2013;2013:605–611. doi: 10.1182/asheducation-2013.1.605. - DOI - PubMed
    1. Parikh S.A., Kapoor P., Letendre L., Kumar S., Wolanskyj A.P. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin. Proc. 2014;89:484–492. doi: 10.1016/j.mayocp.2013.12.012. - DOI - PubMed
    1. Al-Samkari H., Berliner N. Hemophagocytic Lymphohistiocytosis. Annu. Rev. Pathol. 2018;13:27–49. doi: 10.1146/annurev-pathol-020117-043625. - DOI - PubMed

Publication types

LinkOut - more resources