Composite B-cell and T-cell lymphomas: clinical, pathological, and molecular features of three cases and literature review.

Xueli Jin; Hui Liu; Jing LI; Xibin Xiao; Xianggui Yuan; Panpan Chen; Boxiao Chen; Yun Liang; Fengbo Huang

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Composite B-cell and T-cell lymphomas: clinical, pathological, and molecular features of three cases and literature review.

Author: Xueli JIN ¹ ; Hui LIU ² ; Jing LI ³ ; Xibin XIAO ¹ ; Xianggui YUAN ¹ ; Panpan CHEN ¹ ; Boxiao CHEN ⁴ ; Yun LIANG ⁵ ; Fengbo HUANG ⁶
Author Information

1. Department of Hematology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China.
2. Department of Pathology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China.
3. Department of Nuclear Medicine, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China.
4. School of Medicine, Zhejiang University, Hangzhou 310029, China.
5. Department of Hematology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China. liangyun@zju.edu.cn.
6. Department of Pathology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China. 2515183@zju.edu.cn.
Publication Type:Journal Article
Keywords: B-cell lymphoma; Composite lymphoma; T-cell lymphoma
MeSH: Humans; Rituximab/therapeutic use*; Vincristine/therapeutic use*; Prednisone/therapeutic use*; Epstein-Barr Virus Infections/drug therapy*; Herpesvirus 4, Human; Neoplasm Recurrence, Local; Lymphoma, T-Cell/drug therapy*; Cyclophosphamide/therapeutic use*; Lymphoma, Large B-Cell, Diffuse/pathology*; Doxorubicin/therapeutic use*; Lymphadenopathy/drug therapy*; Antineoplastic Combined Chemotherapy Protocols/therapeutic use*
From: Journal of Zhejiang University. Science. B 2023;24(8):711-722
CountryChina
Language:English
Abstract: Composite lymphoma (CL) involving B-cell lymphoma and T-cell lymphoma is extremely rare. Herein, we report three such cases using immunohistochemistry, flow cytometry, and the next-generation sequencing (NGS) to identify the pathological and molecular characteristics of CL. In the first case, the patient was admitted to hospital for generalized pruritic maculopapular rash over the whole body. An excisional biopsy of the skin lesions showed T-cell lymphoma. At the same time, the staging bone marrow (BM) biopsy revealed a diffuse large B-cell lymphoma (DLBCL). After R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapies, the patient produced a good response with substantial dissipation of the rashes and relief of skin. The other two patients were admitted to hospital due to lymphadenopathy and were diagnosed with DLBCL and follicular lymphoma (FL) after core needle biopsy of lymph nodes, BM biopsy, BM aspiration, and flow cytometry. Following R-CHOP and R-COP (rituximab, cyclophosphamide, vincristine, and prednisone) therapies, they achieved complete remission unconfirmed (CRu) and complete remission (CR). However, one or two years later, they suffered a relapse of lymphadenopathy. The shocking fact was that re-biopsy of lymphadenopathy revealed peripheral T-cell lymphoma (PTCL) and angioimmunoblastic T-cell lymphoma (AITL). NGS findings identified DNA methyltransferase 3a (DNMT3a), isocitrate dehydrogenase 2 (IDH2), Ras homolog gene family, member A (RHOA), splicing factor 3B subunit 1 (SF3B1), and tumor protein p53 (TP53) mutations. After immunochemotherapy, these patients achieved CRu and CR again. Nevertheless, they suffered a second relapse of T-cell lymphoma. Finally, they died due to progression of disease. We found that the occurrence of CL is associated with Epstein-Barr virus infection and DNMT3a, IDH2, and TP53 mutations, and the prognosis of the disease is closely related to the T-cell lymphoma components.