Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Hydroa-Vacciniforme like Lymphoproliferative Disorder

  • Atsuko NakazawaEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_3881-1



Hydroa vacciniforme (HV)-like lymphoproliferative disorder (HV-like LPD) is a chronic EBV-positive cutaneous T-cell lymphoproliferative disorder of childhood, associated with the risk of developing systemic lymphoma. HV-like LPD is a polyclonal or (most often) monoclonal disorder of T-cells and/or NK cells, regardless of the presence or absence of systemic symptoms and the severity of the skin lesions. Classic HV, severe HV, and HV-like cutaneous T-cell lymphoma constitute a continuous spectrum of EBV-associated HV-like LPD (Quintanilla-Martinezz et al. 2013, 2017).

Clinical Features

  • Incidence

    HV-like LPD is rare and occurs mainly in children and adolescents from Latin America and East Asia. It is rare in adults. There is a seasonal increased occurrence during the summer (Quintanilla-Martinezz et al. 2013)....

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References and Further Reading

  1. Barrionuevo, C., Anderson, V. M., Zevallos-Giampietri, E., et al. (2002). Hydroa-like cutaneous T-cell lymphoma: A clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. Applied Immunohistochemistry & Molecular Morphology, 10, 7–14.CrossRefGoogle Scholar
  2. Beltran, B. E., Maza, I. N., CB, M.–. A., et al. (2014). Thalidomide for the treatment of hydroa vacciniforme-like lymphoma: Report of four pediatric cases from Peru. American Journal of Hematology, 89, 1160–1161.CrossRefGoogle Scholar
  3. Iwatsuki, K., Satoh, M., Yamamoto, T., et al. (2006). Pathogenic link between hydroa vacciniforme and Epstein-Barr virus–associated hematologic disorders. Archives of Dermatology, 142, 587–595.CrossRefGoogle Scholar
  4. Kimura, H., Ito, Y., Kawabe, S., et al. (2012). EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: Prospective analysis of 108 cases. Blood, 119, 673–686.CrossRefGoogle Scholar
  5. Magana, M., Sangüeza, P., Gil-Beristain, J., et al. (1998). Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): A distinctive type of cutaneous T-cell lymphoma. Journal of the American Academy of Dermatology, 38, 574–579.CrossRefGoogle Scholar
  6. Miyake, T., Yamamoto, T., Hirai, Y., et al. (2015). Survival rates and prognostic factors of Epstein-Barr virus-associated hydroa vacciniforme and hypersensitivity to mosquito bites. The British Journal of Dermatology, 172(1), 56–63.CrossRefGoogle Scholar
  7. Quintanilla-Martinezz, L., Ridaura, C., Nagl, F., et al. (2013). Hydroa vacciniforme-like lymphoma: A chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma. Blood, 122, 3101–3110.CrossRefGoogle Scholar
  8. Quintanilla-Martinezz L, Ko Y-H, Kimura H, et al (2017) Hydroa vacciniforme-like lymphoproliferative disorder. In S. H. Swerdlow, E. Campo, N. L. Harris, et al. (Eds.), WHO classification of Tumours of Haematopoietic and lymphoid tissues (revised 4th ed., pp. 360–362). Lyon: IARC.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Clinical ResearchSaitama Children’s Medical CenterSaitamaJapan