Cutaneous Lymphoproliferative Disorders: What’s New in the Revised 4th Edition of the World Health Organization (WHO) Classification of Lymphoid Neoplasms

Abstract

Cutaneous lymphoproliferative disorders remain a challenging aspect of dermatopathology, in part due to the rarity of the entities and extreme variability in clinical outcomes. Although many of the entities remain unchanged, the approach to some of them has changed in the new 2016 classification scheme of the World Health Organization. Chief among these are Epstein-Barr virus-associated lymphoproliferative disorders such as Epstein-Barr virus-associated mucocutaneous ulcer and hydroa vacciniforme-like lymphoproliferative disorder, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, primary cutaneous acral CD8+ T-cell lymphoma, primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder, and breast implant-associated anaplastic large cell lymphoma. In addition, translocations and gene rearrangements such as those involving the 6p25.3 locus have started to inform diagnosis and classification of anaplastic large cell lymphoma and lymphomatoid papulosis. In this review, we will examine what is new in the diagnostic toolbox of cutaneous lymphoproliferative disorders.

Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases

“…As the disease progressed, most patients exhibited severe, extensive skin lesions with systemic manifestations, including fever, hepatosplenomegaly, and lymphadenopathy, consistent with other reports [2, 19, 20]. The term HV-LPD changed from hydroa vacciniforme-like lymphoma to lymphoproliferative disorder by the 4th revised edition of WHO classifications would be a more appropriate classification and a more descriptive term [1, 21, 22].…”

Epstein–Barr Virus Mucocutaneous Ulcer: An Unexpected Diagnosis of a New Entity

“…Epstein–Barr virus (EBV) mucocutaneous ulcer (MCU) is a rare but underdiagnosed condition, presenting as solitary, sharply demarcated ulcerations of the oral cavities, gastrointestinal tract, and skin [ 1 ]. The disease is a relatively new clinicopathological entity since its inclusion in the 2016 World Health Organisation (WHO) classification of lymphoid neoplasms [ 2 ].…”

“…Usually, atypical Reed–Sternberg-like cells demonstrating CD-15 and CD-30 coexpression while retaining PAX-5 positivity will be present in the polymorphic infiltrate. The dense reactive lymphocytic infiltrates on the periphery of the lesion are usually rich in CD3+ T cells [ 1 – 4 ].…”

Hydroa vacciniforme-like lymphoproliferative disorder in Korea

“…Although patients with HVLPD can show a favorable prognosis, some patients may have significantly serious outcomes, including progression to systemic lymphoma. 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 .…”

“…According to the revised 4th World Health Organization (WHO) classification, the term HVLPD has been renamed from hydroa vacciniforme-like lymphoma to HVLPD owing to its relationship to chronic active EBV infection (CAEBV) and the broad spectrum of its clinical course 1 . Although HVLPD usually affects children, adult and elderly cases have been reported 2 . Clinically, it is characterized by recurrent erythematous papules and vesicles that leave pitted scars on sun-exposed areas, including the face and forearms.…”