CD56‐positive (nasal‐type T/NK cell) lymphoma arising on the skin

Abstract

Several authors have reported cases of patients with malignant lymphoma with unique characteristics, designated nasal-type T/NK cell lymphoma, which expresses the natural killer (NK) cell marker and shows frequent extra-nodal involvement and poor prognosis. We report 2 cases of this type of lymphoma which were CD56-positive and showed a histopathologically angiocentric pattern with cutaneous and subcutaneous tumorous lesions. Patient 1 had extensive invasion of skin, underlying skeletal muscle, spleen and bone marrow, and died of sepsis 34 months after onset. Patient 2 had multiple subcutaneous nodules and invasion to mammary gland, lung, lymph node and spleen at the time of her first visit. She died of a rapid invasion of lymphoma cells to the liver 5 months after onset. Both patients showed similar immunophenotypes of tumor cells (CD2+, CD3-, CD4-, CD8-, CD20-, CD56+) and germ line configuration of the heavy chain of immunoglobulin (JH), T-cell receptor C beta-1 subunit DNA and T-cell receptor J gamma subunit DNA. Epstein-Barr virus early regions RNA was demonstrated in the nuclei of tumor cells of both patients with in situ hybridization. The histopathological examination of the skin lesions of both patients revealed the features of angiocentric lymphoma. The detection of CD56 in the tumor cells of cutaneous lymphomas should be routinely performed for the early diagnosis of this type of lymphoma with extremely poor prognosis.

Blastoid NK Cell Leukemia/Lymphoma with Cutaneous Involvement

“…The nasal-type NK/T cell lymphomas presenting first with cutaneous lesions show the same clinical, histopathological, immunohistochemical and prognostic features than other nasal and nasal-type NK/T cell lymphomas [8, 9]. Cutaneous lesions show a heterogeneous, non-specific picture, with e.g.…”

“…Patients with nasal-type NK/T cell lymphoma present with extranodal disease, usually at multiple sites. The most common site of involvement is the skin, with a frequency of 62% of the cases, with lesions usually being generalized or distributed over multiple anatomical locations [2, 8]. Skin involvement may appear under two forms: at presentation, followed by extracutaneous spread, or as one of several organs involved by generalized lymphoma [9].…”

A Case of Aggressive NK/T-cell Lymphoma/Leukemia with Cutaneous Involvement in Adolescence

“…In the past, these lymphomas were often diagnosed as one of the following entities: lethal midline granuloma, midline malignant reticulosis, lymphoma of large granular lymphocytes, CD56+ angiocentric T-cell lymphoma, and CD56+ hematolymphoid malignancy 3,6,7 . This confusion was caused by the uncertain origin of these uncommon lymphomas.…”

“…This confusion was caused by the uncertain origin of these uncommon lymphomas. Now, these CD56+ lymphomas are better designated as NKTCL because they express NK-cell markers and a limited number of T-cell markers 7 . World Health Organization (WHO) classification subdivided NKTCL into 4 types; extranodal nasal type NKTCL, enteropathy-type T-cell lymphoma, blastic NK-cell lymphoma and aggressive NK-cell leukemia …”

Cytologic Features of NK/T-Cell Lymphoma

“…[21][22][23] This lymphoma also shows a strong association with Epstein-Barr virus (EBV); the virus is demonstrable in almost all cases irrespective of the ethnic origin of the patients. 1,15,[24][25][26] The primary diagnosis and subclassification of lymphomas based on cytologic preparation are of increasing interest as well as a source of much debate. Many papers report on the cytologic diagnosis of lymphomas, especially by fine needle aspiration (FNA) cytology and immunophenotyping by flow cytometry.…”