膿皮症様病変で発症したｃｙｔｏｔｏｘｉｃ ｍｏｌｅｃｕｌｅ，ＣＤ４陽性のαβＴ ｃｅｌｌ ｐｒｉｍａｒｙ ｃｕｔａｎｅｏｕｓ ａｎａｐｌａｓｔｉｃ ｌａｒｇｅ ｃｅｌｌ ｌｙｍｐｈｏｍａの一剖検例

Abstract

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is categorized by CD30-positive atypical tumor cells. It usually shows a good prognosis and is involved in the same spectrum as lymphomatoid papulosis which regresses spontaneously. However, one-fourth of PCALCL cases are known to progress to systemic disease. Histologically, some cases of PCALCL should be differentiated from beneign cutaneous suppurative dermatosis because of neutrophil/lymphocyte-rich coinfiltration.In this study, an autopsied case of PCALCL was presented. The patient was a 72-year-old woman who had complained of the ulcerated cutaneous bulky tumor of the nape for one and a half month. Histologically, CD30-positive atypical large tumor cells with incidental Reed-Sternberg-like cells were observed intermingled with neutrophls, eosinophils and lymphocytes. There was also a wide necrotic area accompanied with medium- sized arteries obliterated with the proliferated endothelial cells and thickend walls. Enlarged cervical lymph nodes were histologically effaced with infiltration of atypical tumor cells and inflammatory cells with fibrosis. Immunohistochemically, tumor cells were positive for CD3, CD4, CD25, Cβ1, granzyme B, TIA-1 and EMA, and negative for CD8, CD15, CD16, CD20, CD56, CD57, p80, Vδ2 and keratin. PCR analysis of paraffin-embedded samples taken from the cutaneous lesion and the lymph node revealed a monoclonal rearrangement band of TCRγ. Six months later, she died of pneumonia, and the autopsy revealed small nodular metastatic lesions in the liver, spleen and bone marrow. From these findings, she was diagnosed as PCALCL with generalized spreading. Necrosis and inflammatory granulation observed in this case might be due to angioobliteratione and/or cytotoxic proteins such as granzyme B and TIA-1 from tumor cells.