Primary central nervous system Hodgkin Lymphoma: A case discussion and a hypothesis on the etiology

Abstract

Hodgkin Lymphoma (HL) is a systemic disease with involvement of the cervical, supraclavicular, and mediastinal lymph nodes. It is commonly diagnosed in patients within the second and third decades of their lives. Diagnosis is usually made based on the distinct morphological and immunohistochemical characteristics, with the tissue biopsy being the cornerstone of workup. Extranodal presentation of HL is unusual and seldom encountered. Primary HL of the central nervous system (CNS) is exceedingly rare. We herein report a case of a 38yearold male patient who was diagnosed with primary CNSHL. The patient was treated with complete surgical resection followed by radiotherapy and chemotherapy. The patient was diseasefree for 7 years postoperatively without any clinical evidence of relapse. We also discussed a possible role of CNS regulatory Tcells (Tregs) in developmental primary CNSHL.

Keywords

Central nervous system lymphoma - cerebellar tumor - Hodgkin Lymphoma - T-regulatory cells

Publication History

Article published online:
09 August 2021

• References

• 1 Re D, Fuchs M, Schober T, Engert A, Diehl V. CNS involvement in Hodgkin’s lymphoma. J Clin Oncol 2007; 25: 3182
• 2 Cecyn KZ, Chaves EM, Oliveira JS. Primary central nervous system involvement in classical Hodgkin’s lymphoma: Case report and review of the literature. J Blood Lymph 2017; 8: 196
• 3 Negi N, Das BK. CNS: Not an immunoprivilaged site anymore but a virtual secondary lymphoid organ. Int Rev Immunol 2018; 37: 5768
• 4 Reynolds AD, Banerjee R, Liu J, Gendelman HE, Mosley RL. Neuroprotective activities of CD4+CD25+ regulatory T cells in an animal model of Parkinson’s disease. J Leukoc Biol 2007; 82: 108394
• 5 Ha TY. The role of regulatory T cells in cancer. Immune Netw 2009; 9: 20935
• 6 Piccirillo CA, Shevach EM. Cutting edge: Control of CD8+ T cell activation by CD4+CD25+ immunoregulatory cells. J Immunol 2001; 167: 113740
• 7 Wolf AM, Wolf D, Steurer M, Gastl G, Gunsilius E, GrubeckLoebenstein B. et al. Increase of regulatory T cells in the peripheral blood of cancer patients. Clin Cancer Res 2003; 9: 60612
• 8 Marshall NA, Christie LE, Munro LR, Culligan DJ, Johnston PW, Barker RN. et al. Immunosuppressive regulatory T cells are abundant in the reactive lymphocytes of Hodgkin lymphoma. Blood 2004; 103: 175562
• 9 Alvaro T, Lejeune M, Salvadó MT, Bosch R, García JF, Jaén J. et al. Outcome in Hodgkin’s lymphoma can be predicted from the presence of accompanying cytotoxic and regulatory T cells. Clin Cancer Res 2005; 11: 146773
• 10 Tzankov A, Meier C, Hirschmann P, Went P, Pileri SA, Dirnhofer S. et al. Correlation of high numbers of intratumoral FOXP3+ regulatory T cells with improved survival in germinal center like diffuse large B cell lymphoma, follicular lymphoma and classical Hodgkin’s lymphoma. Haematologica 2008; 93: 193200
• 11 Badoual C, Hans S, Fridman WH, Brasnu D, Erdman S, Tartour E. et al. Revisiting the prognostic value of regulatory T cells in patients with cancer. J Clin Oncol 2009; 27: e56
• 12 Schreck S, Friebel D, Buettner M, Distel L, Grabenbauer G, Young LS. et al. Prognostic impact of tumourinfiltrating th2 and regulatory T cells in classical Hodgkin lymphoma. Hematol Oncol 2009; 27: 319
• 13 van Blydenstein SA, Patel M, Philip V, Lakha A, Pather S, WestgarthTaylor T. et al. Classical Hodgkin lymphoma involving the central nervous system (brain) an unusual presentation. Clin Case Rep 2014; 2: 8892
• 14 Gerstner ER, Abrey LE, Schiff D, Ferreri AJ, Lister A, Montoto S. et al. CNS Hodgkin lymphoma. Blood 2008; 112: 165861
• 15 Assis MC, Campos AH, Oliveira JS, Soares FA, Silva JM, Silva PB. et al. Increased expression of CD4+CD25+FOXP3+ regulatory T cells correlates with Epstein Barr virus and has no impact on survival in patients with classical Hodgkin lymphoma in Brazil. Med Oncol 2012; 29: 36149