» Articles » PMID: 28228435

Crohn's Disease Complicated by Epstein-Barr Virus-driven Haemophagocytic Lymphohistiocytosis Successfully Treated with Rituximab

Overview
Journal BMJ Case Rep
Specialty General Medicine
Date 2017 Feb 24
PMID 28228435
Citations 6
Authors
Affiliations
Soon will be listed here.
Abstract

We report a case of Epstein-Barr virus (EBV)-driven haemophagocytic lymphohistiocytosis (HLH) in a man with Crohn's disease treated with 6-mercaptopurine and adalimumab therapy who was successfully treated with rituximab therapy alone. This is the first published case in an adult patient with EBV-driven HLH in the setting of thiopurine use and inflammatory bowel disease to be successfully treated with rituximab therapy alone. Here, we will discuss putative immunological mechanisms which may contribute to this potentially life-threatening complication.

Citing Articles

Impact of Epstein-Barr virus infection in patients with inflammatory bowel disease.

Zhang H, Zhao S, Cao Z Front Immunol. 2022; 13:1001055.

PMID: 36389673 PMC: 9651941. DOI: 10.3389/fimmu.2022.1001055.


Hemophagocytic Lymphohistiocytosis and Inflammatory Bowel Disease: Case Report and Systematic Review.

Brambilla B, Barbosa A, Scholze C, Riva F, Freitas L, Balbinot R Inflamm Intest Dis. 2020; 5(2):49-58.

PMID: 32596254 PMC: 7315217. DOI: 10.1159/000506514.


Increased incidence of systemic serious viral infections in patients with inflammatory bowel disease associates with active disease and use of thiopurines.

Wisniewski A, Kirchgesner J, Seksik P, Landman C, Bourrier A, Nion-Larmurier I United European Gastroenterol J. 2020; 8(3):303-313.

PMID: 32529821 PMC: 7184656. DOI: 10.1177/2050640619889763.


Update on the Use of Thiopurines and Methotrexate in Inflammatory Bowel Disease.

Johnson C, Dassopoulos T Curr Gastroenterol Rep. 2018; 20(11):53.

PMID: 30267281 DOI: 10.1007/s11894-018-0658-1.


Hemophagocytic Lymphohistiocytosis Secondary to Unknown Underlying Hodgkin Lymphoma Presenting with a Cholestatic Pattern of Liver Injury.

Booth A, Osehobo P, Rodgers-Soriano D, Lalarukh A, Eltorky M, Stevenson H Case Rep Gastroenterol. 2018; 12(1):99-108.

PMID: 29805352 PMC: 5968292. DOI: 10.1159/000486190.


References
1.
Hugot J, Chamaillard M, Zouali H, Lesage S, Cezard J, Belaiche J . Association of NOD2 leucine-rich repeat variants with susceptibility to Crohn's disease. Nature. 2001; 411(6837):599-603. DOI: 10.1038/35079107. View

2.
Takada H, Ohga S, Mizuno Y, Nomura A, Hara T . Increased IL-16 levels in hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol. 2004; 26(9):567-73. DOI: 10.1097/01.mph.0000134465.86671.2e. View

3.
Green M, Kennell A, Larche M, Seifert M, Isenberg D, Salaman M . Natural killer cell activity in families of patients with systemic lupus erythematosus: demonstration of a killing defect in patients. Clin Exp Immunol. 2005; 141(1):165-73. PMC: 1809425. DOI: 10.1111/j.1365-2249.2005.02822.x. View

4.
Fuss I, Becker C, Yang Z, Groden C, Hornung R, Heller F . Both IL-12p70 and IL-23 are synthesized during active Crohn's disease and are down-regulated by treatment with anti-IL-12 p40 monoclonal antibody. Inflamm Bowel Dis. 2005; 12(1):9-15. DOI: 10.1097/01.mib.0000194183.92671.b6. View

5.
Vacher-Coponat H, Brunet C, Moal V, Loundou A, Bonnet E, Lyonnet L . Tacrolimus/mycophenolate mofetil improved natural killer lymphocyte reconstitution one year after kidney transplant by reference to cyclosporine/azathioprine. Transplantation. 2006; 82(4):558-66. DOI: 10.1097/01.tp.0000229390.01369.4a. View