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    Systemic lymphoma arising from hydroa vacciniforme-like lymphoma: report of two cases with review of literature.

    • Authors:
    • Yu-qiong Yang
      Sichuan University
      Lei Fan
      Jiangsu Province Hospital
      MRs Li Wang, RN
      shepherd university

      walnut , CA | United States
      Ji Xu
      Jiangsu Province Hospital
      Run Zhang
      Jiangsu Province Hospital
      Zheng Ge
      The First Affiliated Hospital of Nanjing Medical University
      Jian-Yong Li
      Jiangsu Province Hospital
      Wei Xu
      The First Affiliated Hospital of Nanjing Medical University
      Nanjing Shi | China
    Int J Clin Exp Pathol 2014 15;7(9):6403-8. Epub 2014 Aug 15.
    Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital Nanjing 210029, China.
    Hydroa vacciniforme-like lymphoma (HVLL) is an extremely rare lymphoma described in children that occurs mainly in Asia and Latin American countries. It is an Epstein-Barr virus (EBV)-positive lymphoproliferative disease (LPD) characterized by a monoclonal proliferation of T or NK cells. In this study, we report the clinical and pathological features of two Chinese patients with HVLL showed T-cell phenotype expressing CD4. The two patients generally presented with similar clinical histories of waxing and waning ulcerative blistering lesions for ten years or more until progression to systemic lymphoma. One patient died two months after progression and another is alive with disease. In the two cases, persistence infection of EBV may be attributed to the disease progression, and systemic lymphoma arising from HVLL behaves in an aggressive fashion and is predisposing to chemotherapeutic agent resistance.
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