F. Poon1,2, E. Ieremia3, E. Soilleux3, G. Collins4, R. Matin1
Angioimmunoblastic T-cell lymphoma (AITL) is a rare nodal peripheral T-cell lymphoma associated with clonal T-cell receptor gene rearrangements. AITL is recognised to result in profound immunosuppression which predisposes to severe infections such as Epstein Barr Virus (EBV). EBV-associated malignancy is rare and presentation is variable. To our knowledge, to date, less than 5 cases of cutaneous EBV-associated diffuse B-cell lymphoma in patients with AITL have been reported.
A 47-year-old male presented with multiple painful abscesses and nodules on bilateral axillae whilst undergoing active curative chemotherapy for AITL. Incisional biopsies of a cutaneous abscess and nodule revealed a diffuse and nodular pan-dermal and subcutaneous atypical lymphoid infiltrate. Immunohistochemistry demonstrated Blymphocytes strongly positive for CD20 with partial loss of CD79a and Pax5, and strongly positive bcl2 and EBVencoded small RNA (EBER). Clonality studies revealed clonal population of T-cells and B-cells. Taking these findings together, in the absence of systemic disease, a diagnosis of cutaneous EBV-positive diffuse large B-cell lymphoma was made.
As a consequence, chemotherapy was changed to salvage R-GDP (gemcitabine, dexamethasone, cisplatin and rituximab). Within 42 days, the cutaneous abscesses and nodules had completely resolved. The patient is currently in partial remission as he completes two further courses of RGDP followed by autologous transplantation.
This case highlights the importance of recognising cutaneous abscesses and nodules in the context of AITL as probable EBV-associated B-cell lymphoma. This distinction is important to ensure that optimal therapeutic strategies are considered for this otherwise relatively aggressive neoplasm.