J Korean Med Sci.  2005 Apr;20(2):319-324. 10.3346/jkms.2005.20.2.319.

CD4+CD56+Lineage Negative Hematopoietic Neoplasm: So Called Blastic NK Cell Lymphoma

• Kim Y
• Kang MS
• Kim CW
• Sung R
• Ko YH

• KMID: 1095212
• DOI: http://doi.org/10.3346/jkms.2005.20.2.319

Abstract

Blastic natural killer (NK) cell lymphoma is a rare neoplasm characterized by blastoid tumor cells expressing CD4 and CD56, with predominant skin involvement. Although this tumor has been regarded as a neoplasm related to NK cell, recent studies suggested that it is derived from plasmacytoid dendritic cells, but not from NK cell. Herein we report 4 cases of CD4+CD56+ lineage marker- blastic NK cell lymphomas with a review of literatures. The patients were 3 men and one woman. Three of them were young (17, 18, and 22 yr old). Three patients had skin lesions, at initial presentation in two patients and during the course of disease in other patient. Histologically, tumors consisted of monotonous medium to large blastoid cells showing no necrosis, angiocentric growth or epidermotrophism. All four tumors were CD4+ and CD56+. Three expressed CD68 antigen. Lineage specific markers for B- and T cell were negative. All tumors did not express myeloperoxidase. T-cell receptor gene rearrangement, EBV, CD13 and CD33 were negative. In one patient, tumor cells arranged in Homer-Wright type pseudorosette and expressed terminal deoxynucleotidyl transferase(TdT). Despite the standard lymphoma chemotherapy, the tumors, except one lost during follow-up, progressed and relapsed. The patients died 8-60 months after diagnosis.

Keyword

Killer Cells, Natural; Lymphoma; Lymphoma, Blastic NK Cell; DNTTIP1 protein, human; Recep-tor-CD3 Complex, Antigen, T-Cell

MeSH Terms

Adolescent
Antigens, CD4/*analysis
Antigens, CD56/*analysis
Cell Lineage
Female
Humans
Killer Cells, Natural/immunology/*pathology
Lymphoma, T-Cell/immunology/*pathology
Male
Middle Aged

Figure

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  Fig. 1 Histologic finding of the skin biopsy (Case 1), dense infiltration of medium-sized blastoid tumor cells without necrosis or angiocentric growth noted in the upper and mid-dermis without epidermotrophism (H&E stain, A; ×100, B; ×250).

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  Fig. 2 In Case 3, tumor cells arranged in Homer-Wright type pseudorosette (A; H&E stain, ×400) with positivity for terminal deoxynucleotidyl transferase (B; ×400).

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  Fig. 3 Results of immunohistochemical stains. Most of tumor cells strongly express CD56 (A) and CD4 (B) (×400).

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