Secondary cutaneous Epstein-Barr virus-associated diffuse large B-cell lymphoma in a patient with angioimmunoblastic T-cell lymphoma: a case report and review of literature

Abstract

Only a few cases of extranodal Epstein-Barr virus (EBV)-associated B-cell lymphomas arising from patients with angioimmunoblastic T-cell lymphoma (AITL) have been described. We report a case of AITL of which secondary cutaneous EBV-associated diffuse large B-cell lymphoma (DLBCL) developed after the initial diagnosis of AITL. A 65-year-old Chinese male patient was diagnosed as AITL based on typical histological and immunohistochemical characteristics in biopsy of the enlarged right inguinal lymph nodes. The patient initially received 6 cycles of chemotherapy with CHOP regimen (cyclophosphamide, vincristine, adriamycin, prednisone), but his symptoms did not disappear. Nineteen months after initial diagnosis of AITL, the patient was hospitalized again because of multiple plaques and nodules on the skin. The skin biopsy was performed, but this time the tumor was composed of large, polymorphous population of lymphocytes with CD20 and CD79a positive on immunohistochemical staining. The tumor cells were strong positive for EBER by in situ hybridization. The findings of skin biopsy were compatible with EBV-associated DLBCL. CHOP-R chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) was then administered, resulting in partial response of the disease with pancytopenia and suppression of cellular immunity. To our knowledge, this is the first case of cutaneous EBV-associated DLBCL originated from AITL in Chinese pepole. We suggest the patients with AITL should perform lymph node and skin biopsies regularly in the course of the disease to detect the progression of secondary lymphomas.

Figure 1

Histopathological findings of lymph node at the initial presentation. (A) The architecture of lymph node was effaced by a diffuse polymorphic infiltration composed of small- to medium-sized lymphoid cells, immunoblastic cells and scattered eosinophils around high-endothelial venules. (B) Multinucleated cells with eosinophilic nucleoli resembling Reed-Stemberg (RS) cells could be observed in the lymph node. (C) Immunohistochemical examination revealed that infiltrated small to medium-sized lymphoid cells were diffusely positive for CD3, but large immunoblast-like cells and RS-like cells were positive for CD20 (D) and CD30 (E).These larger cells were also positive for EBER by in situ hybridization (F).(A, H&E staining, with original magnification ×400; B, H&E staining, with original magnification ×600; C-E, immunohistochemical staining, with original magnification ×400; F, EBER-in situ hybridization, with original magnification ×400).

Figure 2

Histological findings of skin lesion. (A) Low power view of skin lesion showed diffuse infiltration of lymphoid cells in dermal and subcutaneous tissue without epidermotropism. (B) Large atypical lymphoid cells with prominent nucleoli were observed in the skin lesion. Atypical large lymphoid cells were stained positively with CD20 (C) and CD79a (D). However, CD3 positive cells in skin lesion were small lymphocytes with scattered distribution (E). (F) Most of atypical large cells were positive for EBER by in situ hybridization. (A, H&E staining, with original magnification ×40; B, H&E staining, with original magnification ×400; C-E, immunohistochemical staining, with original magnification ×400; F, EBER-in situ hybridization, with original magnification ×400).