Primary hepatic lymphoma as other iatrogenic immunodeficiency-related lymphoproliferative disorders: a case report and review of the literature. (2nd January 2021)

• Record Type: Journal Article
• Authors: Tsuji, Hideaki; Yoshifuji, Hajime; Shindo, Takero; Hishizawa, Masakatsu; Ishida, Ayami; Fujimoto, Masakazu; Kitagori, Koji; Akizuki, Shuji; Murakami, Kosaku; Nakashima, Ran; Hashimoto, Motomu; Tanaka, Masao; Ohmura, Koichiro
• Is Part Of: Modern rheumatology case reports. Volume 5:Number 1(2021)
• Page Start: 172
• Page End: 177
• Publication Date: 2021-01-02
• Subjects: Epstein–Barr virus -- malignant lymphoma -- methotrexate -- lymphoproliferative disorders -- polymyositis; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1080/24725625.2020.1826627
• Languages: English
• ISSNs: 2472-5625

Abstract

Abstract: We report a case of 68-year-old man with stable polymyositis complicated with primary hepatic lymphoma (PHL) as other iatrogenic immunodeficiency-related lymphoproliferative disorders (OIIA-LPD). Multiple liver masses were diagnosed as diffuse large B-cell lymphoma (DLBCL) by biopsy. The LPD was associated with Epstein–Barr virus (EBV) reactivation, because EBV-DNA was detected in peripheral blood, and EBV antigen was detected in the tumour. He presented with high fever, cytopenia and hyperferritinemia, suggesting hemophagocytosis. Only discontinuation of methotrexate and tacrolimus resulted in a dramatic regression of the liver masses and improvement of fever and cytopenia. We review six cases of OIIA-LPD localised in the liver. All cases were DLBCL; 4/6 cases (67%) were positive for EBV staining, and 2/6 cases (33%) were improved after the discontinuation of immunosuppressants. Screening for EBV in blood and liver tumour is important, when a patient in immunosuppressive status presented with liver masses.