Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2021.395
Autopsy and Case Reports
Autopsy Case Report

Herpes simplex virus-1 triggered hemophagocytic lymphohistiocytosis in a patient with granulomatosis with polyangiitis

Vanessa A. States; Meghan E. Kapp

Downloads: 0
Views: 132

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, aggressive hyperinflammatory syndrome in which an inciting event triggers massive, uninhibited activation of T lymphocytes and macrophages. Although viral infections are the most common trigger of HLH, cases of HSV-1 induced HLH are rare in adults. We present the case and postmortem findings of a 27-year-old woman diagnosed with HLH in the setting of immunosuppression for the treatment of granulomatosis with polyangiitis (GPA). Autopsy revealed evidence of herpes simplex virus-1 (HSV-1) infection and no findings suggestive of GPA recurrence.

Keywords

Herpes simplex, Lymphocytes, Lymphohistiocytosis, Hemophagocytic, Macrophages, Vasculitis, Central Nervous System

References

1 George M. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86. http://dx.doi.org/10.2147/JBM.S46255. PMid:24966707.

2 Henter JI, Horne A, Aricó M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48(2):124-31. http://dx.doi.org/10.1002/pbc.21039. PMid:16937360.

3 Campo M, Berliner N. Hemophagocytic lymphohistiocytosis in adults. Hematol Oncol Clin North Am. 2015;29(5):915-25. http://dx.doi.org/10.1016/j.hoc.2015.06.009. PMid:26461151.

4 Liew JW, Jones BL, Hunter AJ. Disseminated herpes simplex masquerading as hemophagocytic lymphohistiocytosis: a case report. Perm J. 2019;23(3):18-202. http://dx.doi.org/10.7812/TPP/18-202. PMid:31167703.

5 Atteritano M, David A, Bagnato G, et al. Haemophagocytic syndrome in rheumatic patients. A systematic review. Eur Rev Med Pharmacol Sci. 2012;16(10):1414-24. PMid:23104659.

6 Drori A, Ribak Y, van Heerden PV, Meir K, Wolf D, Safadi R. Hemophagocytic lymphohistiocytosis due to acute primary herpes simplex virus 1 infection. J Clin Virol. 2015;68:6-10. http://dx.doi.org/10.1016/j.jcv.2015.04.013. PMid:26071327.

7 Honsig C, Beinhardt S, Tomasits J, Dienes HP. Haemophagocytic lymphohistiocytosis associated with fulminant hepatitis and multiorgan failure following primary Epstein-Barr virus and herpes simplex virus type 1 infection. BMJ Case Rep. 2017;2017:bcr2016218310. http://dx.doi.org/10.1136/bcr-2016-218310. PMid:28356254.

8 Cusini A, Günthard HF, Stussi G, et al. Hemophagocytic syndrome caused by primary herpes simplex virus 1 infection: report of a first case. Infection. 2010;38(5):423-6. http://dx.doi.org/10.1007/s15010-010-0037-9. PMid:20602145.

9 Yabushita T, Yoshioka S, Koba Y, et al. Successful treatment of herpes simplex virus (HSV)-1-associated hemophagocytic lymphohistiocytosis (HLH) with acyclovir: a case report and literature review. Intern Med. 2017;56(21):2919-23. http://dx.doi.org/10.2169/internalmedicine.8490-16. PMid:28924117.

10 Frimmel S, Schipper J, Henschel J, Yu TT, Mitzner SR, Koball S. First description of single-pass albumin dialysis combined with cytokine adsorption in fulminant liver failure and hemophagocytic syndrome resulting from generalized herpes simplex virus 1 infection. Liver Transpl. 2014;20(12):1523-4. http://dx.doi.org/10.1002/lt.24005. PMid:25233991.

11 Alidjinou EK, Dewilde A, Terriou L, Lazrek M, Engelmann I, Hober D. Persistent viral DNA detection in blood after primary herpes simplex 1 infection revealed by hepatitis with hemophagocytic syndrome. J Clin Virol. 2015;69:101-3. http://dx.doi.org/10.1016/j.jcv.2015.06.087. PMid:26209389.
 


Submitted date:
11/24/2021

Accepted date:
06/13/2022

Publication date:
08/17/2022

62fcd65da953957b9c3f1c73 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections