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. 2018 Dec;16(6):4772-4778.
doi: 10.3892/etm.2018.6768. Epub 2018 Sep 19.

Clinicopathological analysis of the hydroa vacciniforme-like lymphoproliferative disorder with natural killer cell phenotype compared with cutaneous natural killer T-cell lymphoma

Guan-Nan Wang  1 , Yong Cui  2 , Wu-Gan Zhao  1 , Ling Li  3 , Xu-Dong Zhang  3 , Yu Chang  3 , Xian-Zheng Gao  1 , Ye Li  1 , Ming-Zhi Zhang  3 , Wen-Cai Li  1
Affiliations

Clinicopathological analysis of the hydroa vacciniforme-like lymphoproliferative disorder with natural killer cell phenotype compared with cutaneous natural killer T-cell lymphoma

Guan-Nan Wang et al. Exp Ther Med. 2018 Dec.

Abstract

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is defined as a distinctive clinicopathological type of cutaneous lymphoma and a subset of patients with this disease exhibit the natural killer (NK)-cell phenotype. The HVLPD-NK cell phenotype may be difficult to distinguish from cutaneous natural killer T-cell lymphoma (CNKTL), as these two diseases share similar immunophenotypic markers. Therefore, the aim of the present study was to analyze the clinicopathological features of this rare disease and compare these features with those of CNKTL. The clinical, histopathological and molecular features of 5 patients with the HVLPD-NK cell phenotype and 11 patients with CNKTL were evaluated. As well as certain subtle histopathological differences, there marked differences the age, distribution of lesions and clinical course differed between patients with these two diseases. These results suggest that the HVLPD-NK cell phenotype should be classified as a separate disorder and treated accordingly.

Keywords: Epstein-Barr virus; cluster of differentiation 56; cutaneous T cell lymphoma; differential diagnosis; hydroa vacciniforme-like lymphoproliferative disorder; natural killer T-cell lymphoma.

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Figures

Figure 1.
Figure 1.
Case 4. Ulceration and scars on the face with marked edema.
Figure 2.
Figure 2.
Case 5. (A) Squamous epithelium was not infiltrated by tumor cells (H&E staining, magnification, ×50). (B) Lymphoid cells were small-medium in size, mixed with some plasma cells and neutrophils (H&E staining, magnification, ×200). Neoplastic cells were positive for (C) CD56 and (D) CD30 (immunoperoxidase with hematoxylin counterstain; magnification, ×400). CD, cluster of differentiation; H&E, hematoxylin and eosin.
Figure 3.
Figure 3.
Histopathological features of cutaneous natural killer/T-cell lymphoma. (A) Skin adnexa involvement by large sized cells with marked atypia (H&E staining, magnification, ×100). (B) Coagulative necrosis within the subcutaneous tissue (H&E staining, magnification, ×100). H&E, hematoxylin and eosin.
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