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Long-term remission in a child with refractory EBV+ hydroa vacciniforme-like T-cell lymphoma through sequential matched EBV+-related allogeneic hematopoietic SCT followed by donor-derived EBV-specific cytotoxic T-lymphocyte immunotherapy

Bone Marrow Transplantation volume 46, pages 759–761 (2011)Cite this article

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The EBV+ T-cell lymphoproliferative disease (T-LPD) of childhood was newly incorporated into the 2008 World Health Organization classification of lymphoid neoplasms. Two types of EBV-associated T-cell malignancies are included in this category: systemic EBV+ T-LPD of childhood and hydroa vacciniforme (HV)-like lymphoma.1 HV-like lymphoma is a peripheral T-cell lymphoma manifested by dermal lesions in mostly sun-exposed areas. Lesions are papulovesicular with ulceration, crusting and edematous changes that leave residual scars. Patients have a backdrop of chronic active EBV infection that usually affects T lymphocytes.2 Refractory to chemotherapy, HV-like lymphoma is a smoldering yet fatal disease.3 Allogeneic hematopoietic SCT (allo-SCT) offers the only established curative option.4 Recent developments in EBV-specific CTL immunotherapeutics may offer an exciting novel treatment approach for this high-risk group of patients. EBV-CTL immunotherapy has demonstrated efficacy in preventing the onset of type III latency EBV+ LPD when given as prophylaxis to >100 allo-SCT recipients and has induced sustained CRs in 11 of 13 patients with EBV+ B-cell post-transplant LPD.5

We report on an Ecuadorian male who presented at age 11 years with a 3-year history of recurrent papulovesicular rash on sun-exposed skin that would spontaneously heal leaving pitting scars. At the time of presentation, these lesions were persistent, widespread, and associated with systemic symptoms. Skin biopsy revealed spongiosis, necrosis and a deep atypical CD3+/CD8+/EBER+ cytotoxic T-cell infiltrate. PCR analysis demonstrated monoclonal TCR-γ gene rearrangement in the skin biopsy, peripheral blood and BM. EBV PCR and serology testing were strongly positive while liver enzyme and lactate dehydrogenase levels were elevated. Computed tomography scan was originally normal; however, positron emission tomography (PET) scan revealed cervical adenopathy. BM and cerebrospinal fluid studies did not show overt morphologic evidence of disease. On the basis of these findings, a diagnosis of systemic EBV+ HV-like T-cell lymphoma was established.

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References

  1. Lim MS, De Leval L, Quintanilla-Martinez L . Commentary on the 2008 WHO classification of mature T- and NK-cell neoplasms. J Hematopathol 2009; 2: 65–73.

    Article  Google Scholar 

  2. Cohen JI, Kimura H, Nakamura S, Ko YH, Jaffe ES . Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts: a status report and summary of an international meeting, 8-9 September 2008. Ann Oncol 2009; 20: 1472–1482.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F et al. Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. Appl Immunohistochem Mol Morphol 2002; 10: 7–14.

    PubMed  Google Scholar 

  4. Sato E, Ohga S, Kuroda H, Yoshiba F, Nishimura M, Nagasawa M et al. Allogeneic hematopoietic stem cell transplantation for Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative disease in Japan. Am J Hematol 2008; 83: 721–727.

    Article  PubMed  Google Scholar 

  5. Bollard CM, Gottschalk S, Leen AM, Weiss H, Straathof KC, Carrum G et al. Complete responses of relapsed lymphoma following genetic modification of tumor-antigen presenting cells and T-lymphocyte transfer. Blood 2007; 110: 2838–2845.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Corradini P, Dodero A, Zallio F, Caracciolo D, Casini M, Bregni M et al. Graft-versus-lymphoma effect in relapsed peripheral T-cell non-Hodgkin's lymphomas after reduced-intensity conditioning followed by allogeneic transplantation of hematopoietic cells. J Clin Oncol 2004; 22: 2172–2176.

    Article  Google Scholar 

  7. Focosi D, Caracciolo F, Galimberti S, Papineschi F, Petrini M . False positive PET scanning caused by inactivated influenza virus vaccination during complete remission from anaplastic T-cell lymphoma. Ann Hematol 2008; 87: 343–344.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, NewYork-Presbyterian Morgan Stanley Children's Hospital, Columbia University, New York, NY, USA

    N K El-Mallawany & M S Cairo

  2. Department of Dermatology, Kravis Children's Hospital at Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA

    L Geller

  3. Department of Pediatrics, Center for Cell and Gene Therapy, Baylor College of Medicine, Methodist Hospital and Texas Children's Hospital, Houston, TX, USA

    C M Bollard

  4. Department of Pediatric Hematology and Oncology, Kravis Children's Hospital at Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA

    B Wistinghausen

  5. Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, NIH, Bethesda, MD, USA

    F Mussai & A S Wayne

  6. Department of Medicine, NewYork-Presbyterian Morgan Stanley Children's Hospital, Columbia University, New York, NY, USA

    B Alobeid & M S Cairo

  7. Department of Pathology and Cell Biology, NewYork-Presbyterian Morgan Stanley Children's Hospital, Columbia University, New York, NY, USA

    M S Cairo

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  1. N K El-Mallawany
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  2. L Geller
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  3. C M Bollard
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  7. B Alobeid
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  8. M S Cairo
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Correspondence to M S Cairo.

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El-Mallawany, N., Geller, L., Bollard, C. et al. Long-term remission in a child with refractory EBV+ hydroa vacciniforme-like T-cell lymphoma through sequential matched EBV+-related allogeneic hematopoietic SCT followed by donor-derived EBV-specific cytotoxic T-lymphocyte immunotherapy. Bone Marrow Transplant 46, 759–761 (2011). https://doi.org/10.1038/bmt.2010.176

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