A Case of CD30+ Nasal Natural Killer/T-Cell Lymphoma

Abstract

Extranodal nasal natural killer (NK)/T-cell lymphoma is a very rare lymphoma characterized by strong association with Epstein-Barr virus infection, very aggressive clinical behavior, and poor prognosis. The typical phenotype of neoplastic natural killer cells in this entity is as follows: CD2+, CD56+, surface CD3-, cytoplasmic CD3epsilon+, and cytotoxic granule-associated protein positive. CD30 expression, a phenotype characteristic of anaplastic large-cell lymphomas, is not a typical feature of nasal NK/T-cell lymphomas. We describe the case of a 42-year-old woman with chronic nasal congestion and septal deviation who presented with progressive generalized tender erythematous plaques. A skin biopsy revealed an atypical angiocentric mononuclear cell infiltrate. Strong CD30 and CD3e immunoreactivities were noted in large atypical mononuclear cells within the infiltrate initially suggestive of a CD30+ T-cell lymphoma. However, flow cytometry of the skin lesion indicated that the cells were CD2+, CD4-, CD8-, and lacked surface CD3 more typical of a neoplasm of natural killer cells. Further studies revealed that the cells were CD56+, T-cell-restricted intracellular antigen-1+, and contained Epstein-Barr virus sequences consistent with a nasal-type NK/T-cell lymphoma. High titers of Epstein-Barr virus in the blood, evidence of sinonasal disease, and absence of a T-cell receptor gene rearrangement were additional features consistent with the diagnosis. The patient had a very aggressive clinical course and, despite combination chemotherapy, died 8 months after the onset of skin lesions. This case represents an example of nasal-type NK/T-cell lymphoma with expression of CD30. When presenting in the skin, the phenotypic and morphologic features of this lymphoma may lead to an erroneous diagnosis of a CD30+ large-T-cell lymphoma.

Adrenal extranodal NK/T‐cell lymphoma diagnosed by fine‐needle aspiration and cerebrospinal fluid cytology and immunophenotyping: A case report

“…However, expression of CD30 in extranodal NK cell lymphoma of nasal type has been reported. 22 Immunohistochemical analysis of 84 cases diagnosed as nasal-type NKTCL showed CD30 expression in 35% of tumors. 23 Expression of CD30 has been shown to correlate with cellular pleomorphism in NKTCL.…”

An extraordinary T/NK lymphoma, nasal type, occurring primarily in the prostate gland with unusual CD30 positivity: case report and review of the literature

“…Expression of CD30, an important marker for anaplastic large-cell lymphomas, in NKTCL is rare. One case of CD30+ NKTCL occurring on skin was reported in 2008 [12]. In that case, Strong CD30, CD3ϵand CD56 immunoreactivities were noted in large atypical mononuclear cells.…”

Angioinvasive Lymphomatoid Papulosis

“…26,27 The histologic findings of angiocentric and angiodestructive infiltrates of atypical lymphocytes and their cytotoxic CD8 + phenotype in most cases of LyP type E may result in interpretation of these cases as highly malignant and aggressive lymphomas. [28][29][30][31][32][33][34][35][36] With regard to the low-malignant course with the proclivity of the lesions to undergo spontaneous regression and the excellent prognosis, it is crucial to differentiate this LyP variant from primary cutaneous lymphomas with angiocentric infiltrates and/or a cytotoxic CD8 + or CD56 + phenotype. In the majority of cytotoxic CD8 + cutaneous T-cell lymphomas, the course of the disease is characterized by rapid progression with cutaneous and visceral spread and fatal outcome within months to few years.…”