Track: Case Reports

Topic: Lymphoproliferative Disorders

Abstract:

Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive subtype of peripheral T-cell lymphoma typically characterized by B symptoms, lymphadenopathy, and myriad cutaneous findings. This report details AITL in a 54-year-old female presenting as erythroderma. The initial clinical presentation and histopathologic evaluation at an outside hospital was consistent with mycosis fungoides (MF). Despite numerous first line therapies for MF, she demonstrates minimal response and was referred to a tertiary care center for additional management recommendations. At presentation to our facility, cutaneous biopsy revealed small to medium sized hyperchromatic and convoluted intraepidermal lymphocytes, arranged in clusters and single cells. The superficial dermis displayed a dense infiltrate of similar cells in a band-like distribution. In the dermis, the infiltrate extended into the adnexa with intrafollicular lymphocytes and increased mucin deposition, supportive of MF with folliculotropism. PET-CT imaging revealed metabolically active lymph nodes and an excisional nodal biopsy was consistent with AITL. Re-evaluation of the cutaneous biopsy with additional immunohistochemistry identified a follicular helper cell phenotype consistent with cutaneous involvement by AITL. This case emphasizes the importance of a comprehensive work up for diagnosis and treatment selection. Early recognition and multidisciplinary management are essential for optimal outcomes in AITL patients.

Presentation Poster

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Pamela Allen, MD