Hydroa Vacciniforme-like Lymphoproliferative disorder in an adult invades the liver and bone marrow with clear pathological evidence: a case report and literature review

BMC Infect Dis. 2021 Jan 6;21(1):17. doi: 10.1186/s12879-020-05697-x.

Abstract

Background: Hydroa Vacciniforme-like Lymphoproliferative Disorder (HV-LPD) is the name given to a group of Epstein-Barr virus (EBV)-associated diseases. It resembles hydroa vacciniforme (HV), the rarest form of photosensitivity, and is a T-cell disorder associated with an Epstein-Barr virus infection. The majority of diagnosed cases occur in East Asia and South America. It is rare in the United States and Europe. Multiple studies have revealed the clinical manifestation of an enlarged liver, but no gold standard such as pathology has yet supported this as a clinical sign of HV-LPD.

Case presentation: Here, we report a case of a 34-year-old Asian female with definite liver invasion. The patient had complained of a recurring facial rash for many years. The patient was admitted to the hospital because of an enlarged liver. After hospitalization, she was given an EB virus nucleic acid test. The EB virus nucleic acid test was positive, and pathological examination suggested that HV-LPD had invaded the skin, bone marrow, and liver. After being given antiviral treatment, the patient's symptoms were mitigated.

Conclusions: Our case confirms the liver damage was caused by HV-LPD and the effectiveness of antiviral treatment.

Keywords: Case report; Hydroa Vacciniforme-like Lymphoproliferative disorder.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Antiviral Agents / therapeutic use
  • Beijing
  • Bone Marrow / pathology*
  • Bone Marrow / virology
  • Epstein-Barr Virus Infections / complications*
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / drug therapy
  • Epstein-Barr Virus Infections / virology
  • Exanthema / complications
  • Exanthema / drug therapy
  • Female
  • Hepatomegaly / drug therapy
  • Hepatomegaly / virology
  • Herpesvirus 4, Human / genetics*
  • Humans
  • Hydroa Vacciniforme / complications*
  • Hydroa Vacciniforme / diagnosis*
  • Hydroa Vacciniforme / drug therapy
  • Hydroa Vacciniforme / pathology
  • Liver / pathology*
  • Liver / virology
  • Lymphoma, T-Cell / complications
  • Lymphoma, T-Cell / drug therapy
  • Lymphoma, T-Cell / virology
  • Lymphoproliferative Disorders / complications*
  • Lymphoproliferative Disorders / diagnosis*
  • Lymphoproliferative Disorders / drug therapy
  • Lymphoproliferative Disorders / pathology
  • Skin / pathology
  • Treatment Outcome

Substances

  • Antiviral Agents