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

Abstract

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.

Keywords: Epstein-Barr virus infections; Hydroa vacciniforme; lymphoma.

Figure 1

Morphology, immunophenotype and in situ hybridization (ISH) for EBV-encoded small RNA (EBER) of HVLL (Case 1). A. Skin biopsy showing an infiltrate mainly in the dermis that extends into the subcutaneous fat. Note that the infiltrate surrounds the vascular. (Haematoxylin and eosin [H&E], original magnification stain×40); B. Higher power examination shows small-to-medium-sized lymphoid infiltrate without atypia surrounding a blood vessel. (H&E stain, original magnification×100); C. CD3 staining reveals many positive cells. (immunoperoxidase, original magnification×400); D. CD4 stain shows that the cells surrounding the vascular are CD4 positive. (immunoperoxidase, original magnification×400); E. EBER ISH is positive in the infiltrating lymphocytes. (original magnification×400).