Acta Dermato-Venereologica 98-10CompleteContent | Page 17

981 SHORT COMMUNICATION Secondary Cutaneous Epstein-Barr Virus-associated Diffuse Large B-cell Lymphoma with Hodgkin/ Reed-Sternberg-like Cells in a Patient with Angioimmunoblastic T-cell Lymphoma Hao CHEN 1# , Yan-ning XUE 2# , Chong-mei XIN 3 , Jing-shu XIONG 1 , Xiao NI 4 and Jian-Fang SUN 1,5 * 1 Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, 12 Jiangwangmiao Road, Nanjing, 210042, Jiangsu, Departments of Dermatology: 2 Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, Jiangsu, 3 Affiliated Zhongshan Hospital of Fudan University, Shanghai, China and 4 The University of Texas MD Anderson Cancer Center, Houston, USA, and 5 Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs, Nanjing, Jiangsu, China. *E-mail:[email protected] # These authors contributed equally to this work. Accepted Jun 26, 2018; Epub ahead of print Jun 29, 2018 The co-existence of 2 different types of lymphoma in a single patient is rare. We report here a case of secondary cu- taneous Epstein-Barr virus (EBV)-associated diffuse large B-cell lymphoma (DLBCL) with Hodgkin/Reed-Sternberg- like (HRS-L) cells that developed in a patient with lymph node angioimmunoblastic T-cell lymphoma (AITL). CASE REPORT A 49-year-old Chinese woman presented with enlarged left cervical lymph nodes for 2 months in September 2010. She had no visible lesions on her skin. Computed tomography (CT) revealed enlarged bilateral cervical lymph nodes and hepatosplenomegaly. Lymph node biopsy revealed that the normal lymph node architecture was destroyed by small- to medium-sized lymphoid cells with clear cytoplasm and mildly irregular nuclei (Fig. 1A). Plasma cells, eosinophils and scattered large immunoblast-like lymphoid cells were observed. Those small- to medium-sized lymphoid cells were positive for CD3, CD4, CD10, PD-1, and CXCL-13, but negative for CD20 and CD30. The scattered large immunoblast-like cells were positive for CD20 and weakly positive for CD30. The proli- feration of follicular dendritic cells was highlighted by CD21 and CD23 staining. No EBER-positive cells were observed on an in situ hybridization assay. A diagnosis of AITL was made according to World Health Organization (WHO) classification criteria (1). The patient received 6 cycles of chemotherapy with CHOP regimen (cyclophosphamide, doxyrubin, vincristine, prednisone), which led to complete remission. She was followed-up for 21 months without treatment, with no further symptoms. In June 2012, the patient developed multiple plaques and nodu- les, with ulcers on her trunk and extremities (Fig. 1B). The lesions had nodal and perivascular proliferation of small- to medium-sized lymphocytes in the dermal and upper subcutis, with scattered large-sized atypical lymphoid cells. The small- to medium-sized lymphocytes were positive for CD3 and CD4, but negative for CD10, CD20, PD-1 and CXCL-13. The large atypical cells were positive for CD20 and weakly positive for CD30, and negative for CD3, CD4 and CD10. The patient was diagnosed with cutaneous lymphoid atypical proliferation. After diagnosis, the patient was treated with prednisone, 30 mg/ day for 3 weeks, and most of the lesions disappeared. However, one nodule on the chest persisted and enlarged after 2 months. A second biopsy of the chest nodule revealed diffuse proliferation of medium-to-large-sized atypical lymphoid cells in the dermal and subcutis, mixed with numbers of HRS-L large cells (Fig. 1C, D). The medium-to-large-sized cells expressed CD20 (Fig. 1E), CD79a, Bcl-2, Bcl-6 and MUM-1. The HRS-L cells expressed CD20 in varying degrees (Fig. 1F). Both of the populations of cells were negative for CD3, CD5 and CD10. In addition, the HRS-L cells were positive for CD30 (Fig. 1G), but not for CD15 and ALK. EBER were positive in 2 populations of cells. Based on these findings, a diagnosis of EBV+DLBCL was made. After reviewing all 3 biopsies, scattered atypical large-sized cells were found in the first skin biopsy. These cells weakly expressed Fig. 1. (A) Lymph node biopsy revealed that the normal architecture of the lymph node was destroyed by small- to medium-sized lymphoid cells with clear cytoplasm and mildly irregular nuclei. There was marked proliferation of high endothelial venules (haematoxylin and eosin (H&E), ×20). (B) Multiple plaques and nodules with ulcers and crusts on the back. (C) Tumour cells diffusely infiltrated in the dermis (H&E ×5). (D) Large polygonal cells, resembling Hodgkin/Reed-Sternberg-like (HRS-L) large binuclear cells (red arrows) and mononuclear cells (black arrows) or hallmark cells of anaplastic cell lymphoma (green arrows). Some cells have bizarre pleomorphic nuclei (yellow arrows) (H&E ×80). (E) Immunohistochemical staining showed that the medium- to large-sized cells expressed CD20 (×40). (F) Immunohistochemical staining showed that the HRS-L cells were positive for CD20 to varying degrees (black arrow: strong; red arrows: negative; yellow: mild) (×40). (G) Immunohistochemical staining showed that the HRS-L cells were positive for CD30 (×40). This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica. doi: 10.2340/00015555-2996 Acta Derm Venereol 2018; 98: 981–982