Hydroa vacciniforme‐like lymphoproliferative disorder in an elderly Chinese patient and a literature review of adult cases

Abstract

Hydroa vaccinforme-like lymphoproliferative disorder (HVLPD) is a rare T-cell lymphoproliferative disorder driven by the Epstein-Barr Virus (EBV). It was initially designated under the 2008 WHO classification of tumors of hematopoietic and lymphoid tissues as a distinct entity but has been recently reclassified as a lymphoproliferative disorder under the 2016 revision of the 2008 WHO classification of lymphoid malignancies. HVLPD mainly affects Asian, Central, and South American children. It is rarely seen in adults. Although HVLPD may initially be limited to cutaneous involvement and run an indolent course, some patients may develop a rapidly aggressive course. We report a case of an elderly Chinese man with cutaneous-limited disease and a favorable course. A review of adult cases of HVLPD published in the English literature, with comparison to our case, is also presented in this paper.

Hydroa vacciniforme-like lymphoproliferative disorder in Korea

“…These cases with extracutaneous involvement of atypical lesional cells are diagnosed as systemic EBV+ T-cell lymphoma of childhood according to the revised 4th WHO classification 1 , 2 . Although classic HVLPD usually occurs in the pediatric age group, there have been cases of systemic EBV+ T-cell lymphoma in adults with HVLPD 3 , 5 , 22 .…”

EBV associated T‐ and NK‐cell lymphoproliferative diseases: A comprehensive overview of clinical manifestations and novel therapeutic insights

“…69,75,93 Furthermore, several studies have found that patients who get chemotherapies have a worse prognosis than those who do not. 73,92 Most of the data available in this field considered the CHOP regimen as the main therapy, and as we explained earlier, CHOP is an anthracycline-based regimen that is not effective in F I G U R E 2 Treatments for EBV associated LPDs. Four types of treatments are suggested to be effective against EBV associated LPDs: chemotherapy, immunotherapy, targeted therapy and hematopoietic stem cell transplantations.…”

“…These three subtypes of EBV NK/T LPDs, which have close relevance, have not responded well to chemotherapies. 13,71,73,32,92 Unfortunately, due to the rarity of these disorders, finding an effective therapy has been difficult. No effective treatments, except HSCT, have been suggested for patients with sys CAEBV, HV, and SMBA.…”

“…2‐year OS in patients with systemic involvement has been 36%. In addition, several studies have reported that higher levels of EBV‐related antibody titres predict poorer prognosis 70,73–75 …”

“…Studies have shown that chemotherapy has almost no benefit in these patients, and with the modest number of cases with response to the regimen, the chances of refraction or relapse are extremely high 69,75,93 . Furthermore, several studies have found that patients who get chemotherapies have a worse prognosis than those who do not 73,92 . Most of the data available in this field considered the CHOP regimen as the main therapy, and as we explained earlier, CHOP is an anthracycline‐based regimen that is not effective in treating these disorders.…”