Intestinal ulcers as an initial finding in EBV-associated lymphoproliferative disorder

Abstract

Rationale: Epstein-Barr virus (EBV)-associated T-cell lymphoproliferative disorder (LPD) usually occurs in children and young adults. Gastrointestinal involvement is rare. EBV-associated T-cell lymphoproliferative disorder manifesting as intestinal ulcers poses diagnostic challenges clinically and pathologically because of the atypical manifestations. We concluded that some indicators according to our case and literatures, which might be helpful to the diagnosis of EBV-associated LPD manifested as intestinal ulcers. Patient concerns: Here we present a 26-year-old man with complaints of diarrhea and abdominal pain that had persisted for 1 year. Multiform and multifocal deep ulcers were discovered in the colonoscopy. Cell atypia was not obvious but colitis with crypt distortion was found in pathology. Diagnoses: According to the symptoms, laboratory examinations, colonoscopy and pathology results, Crohn Disease was diagnosed. Interventions Infliximab therapy was initiated based on the diagnosis of Crohn Disease. Outcomes: After the fifth course of therapy, intermittent fever and hematochezia occurred. Physical examination revealed swollen tonsils and ulcers, and purulent exudate from the right tonsil and palatoglossal arch were observed. Biopsies obtained through colonoscopy and nasopharyngoscopy demonstrated EBV-associated T-cell proliferation disease (level 3). After that, the tissue sample from the first colonoscopy was reexamined immunohistochemically. The result suggested EBV-associated T-cell proliferation disease (level 1). Lessons: When we confront with patients with multiform and multifocal deep intestinal ulcers, not only the common diseases such as Crohn Disease and intestinal tuberculosis should be considered, EBV-associated T-cell proliferation disease should be considered as well. Repeated multiple biopsy, gene rearrangement, EBV DNA quantitative analysis result, EBV-encoded RNA(EBER) and experienced pathologists might be helpful to the diagnosis.

Clinical features of NK/T-cell EBV-associated LPD manifested as gastrointestinal symptoms in patients with normal immunity: a case report and literature review

“…The most common clinical manifestation in patients with EBV + T/NK-LPD was fever (the temperature ranged from 37 to 41 °C) [ 1 ]. However, not all the patients had a fever; some patients developed fever after applying hormonal or anti-TNF [ 6 ]. Besides fever, diarrhea, abdominal pain, and hematochezia were also observed.…”

“…Briefly, the clinical manifestations of EBV + T/NK LPD, which manifested as gastrointestinal symptoms are atypical. Therefore, most patients were misdiagnosed with CD, infection, and so on [ 3 , 6 , 7 ].…”

“…However, liver dysfunction was observed in 77% EBV + T/NK LPD patients [ 11 ]; which may be related to the different initial infiltrating positions. Deep lymphadenopathy in CT was also commonly reported [ 3 , 4 , 6 , 10 , 11 ]; however, superficial lymphadenopathy and hepatosplenomegaly were rare [ 4 ]. According to Kimura et al [ 1 ], hepatomegaly and lymphadenopathy are common in patients with EBV-associated T/NK-lymphoproliferative cell diseases.…”

“…Yet, there is still some other non-typical behavior in colonoscopies, such as diffusely atrophic small-intestinal villi and erythema [ 4 , 12 ]. As for CT examination, segmental wall thickening was common [ 6 , 11 ], which was similar to CD and ITB.…”

“…Currently, there is limited available literature on this matter that is mostly based on sporadic case reports, most of which were misdiagnosed [ 3 , 5 , 6 ]. This disease shows a fulminant clinical course with poor prognosis, and relying solely on mucosal biopsy for diagnosis is not sufficient.…”