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Disappearance Of Bilateral Adrenal Tumours: Immunodeficiency-associated Lymphoproliferative Disorder In A Patient With Rheumatoid Arthritis

Hanai, S. 2021Adrenal tumor

Hanai, S., Kobayashi, K., Kawashima, I., Ichijo, M., & Nakagomi, D. (2021). Disappearance of bilateral adrenal tumours: immunodeficiency-associated lymphoproliferative disorder in a patient with rheumatoid arthritis. Rheumatology (Oxford, England), 60(7), e255–e257. https://doi.org/10.1093/rheumatology/keab138

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Case Details

Disease Location

Adrenal gland

Personal Characteristics

72-year-old japanese woman. 13-year history of rheumatoid arthritis (ra) and had received oral methotrexate (mtx) and infliximab for 12 years: the doses of mtx and infliximab at the time of presentation were 8 mg/week and 3 mg/kg/ 8 weeks

Clinical Characteristics

Presented with low back pain for over 2 months. Contrast-enhanced CT showed bilateral adrenal tumours; the right tumour was huge and invaded the liver and the right kidney. Ultrasonography and CT-guided percutaneous transhepatic tumour biopsy were performed. The biopsy specimen showed diffuse infiltration of large atypical lymphoid cells. Positive for CD20 on immunohistochemistry. In situ hybridization for ebv-encoded small RNA detected some positive cells. And the patient was diagnosed with ebv-positive diffuse large b-cell lymphoma (dlbcl). Mtx and infliximab were discontinued given the possibility of other iatrogenic immunodeficiency-associated lymphoproliferative disorder (oiia-lpd) associated with mtx and tnfi.

Remission Characteristics

Six months after the biopsy, the tumours regressed markedly bilaterally, and complete remission was observed on PET combined with CT

Treatment & Mechanisms

Proposed Remission Mechanisms

Ebv-positive dlbcl in the adrenal gland, might have contributed to the lpd regression

Clinical Treatment

Biopsy, mtx, and tnfi withdrawal