Yamashita Y, Tsuzuki T, Nakayama A, Fujino M, Mori N
First Department of Pathology, Nagoya University School of Medicine, Japan.
Pathol Int. 1999 Mar;49(3):241-6. doi: 10.1046/j.1440-1827.1999.00854.x.
A case of nasal type natural killer (NK)/T cell lymphoma of the subcutis showing clinical and morphological features that resemble subcutaneous panniculitis-like T cell lymphoma (SPTCL) is presented. A 73-year-old man presented with swelling of the left arm and was diagnosed with panniculitis by a dermatologist. It was concluded from a skin biopsy specimen that the patient had non-Hodgkin's lymphoma of the large cell, NK/T cell type because the neoplastic cells showed polyclonal CD3 immunoreactivity. Treatment with interferon-gamma was initiated, but the patient died of disseminated intravascular coagulation and multiple organ failure 2 months after the initial symptoms appeared. However, involvement of additional organs by the lymphoma was not apparent clinically. An autopsy was not performed. A routinely stained section of the biopsy skin specimen revealed massive necrosis of the subcutaneous fat, karyorrhexis admixed with reactive histiocytes, and large atypical lymphoid cells. Immunoreactivity for polyclonal CD3 was present in the perinuclear region, but absent in the neoplastic cell membranes. CD56, CD45RO (UCHL-1), CD43 (MT1), CD45 (leukocyte common antigen), and the cytotoxic molecules perforin, granzyme B and TIA-1 were positive, but CD20 (L26), CD4, CD8, and betaF1 were negative. Epstein-Barr virus (EBV) mRNA was detected in the nuclei of neoplastic cells by in situ hybridization. Subcutaneous panniculitis-like T cell lymphoma is reported to be an EBV-negative, clonal T cell neoplasm. Although this case showed clinical and morphological features that resembled SPTCL, perinuclear polyclonal CD3 staining and membranous CD56 reactivity seen in neoplastic cells were suggestive of NK cells. Furthermore, the neoplastic cells were positive for EBV. This case is considered to be a NK/T cell lymphoma of the subcutis resembling SPTCL. It is believed that it is important to recognize such a tumor because patients may undergo a fulminant clinical course, despite the tumor being localized in the subcutaneous adipose tissue.
本文报告1例皮下鼻型自然杀伤(NK)/T细胞淋巴瘤,其临床及形态学特征类似于皮下脂膜炎样T细胞淋巴瘤(SPTCL)。1名73岁男性因左臂肿胀就诊,皮肤科医生诊断为脂膜炎。皮肤活检标本显示患者为大细胞NK/T细胞型非霍奇金淋巴瘤,因为肿瘤细胞显示多克隆CD3免疫反应性。开始使用干扰素-γ治疗,但患者在出现初始症状2个月后死于弥散性血管内凝血和多器官功能衰竭。然而,临床上未发现淋巴瘤累及其他器官。未进行尸检。活检皮肤标本的常规染色切片显示皮下脂肪大量坏死,核碎裂并伴有反应性组织细胞,以及大的非典型淋巴细胞。多克隆CD3免疫反应性存在于核周区域,但肿瘤细胞膜上不存在。CD56、CD45RO(UCHL-1)、CD43(MT1)、CD45(白细胞共同抗原)以及细胞毒性分子穿孔素、颗粒酶B和TIA-1呈阳性,但CD20(L26)、CD4、CD8和βF1呈阴性。原位杂交检测到肿瘤细胞核内存在爱泼斯坦-巴尔病毒(EBV)mRNA。据报道,皮下脂膜炎样T细胞淋巴瘤是一种EBV阴性的克隆性T细胞肿瘤。尽管该病例显示出类似于SPTCL的临床和形态学特征,但肿瘤细胞中可见的核周多克隆CD3染色和膜性CD56反应性提示为NK细胞。此外,肿瘤细胞EBV呈阳性。该病例被认为是1例类似于SPTCL的皮下NK/T细胞淋巴瘤。尽管肿瘤局限于皮下脂肪组织,但患者可能会经历暴发性临床病程,因此认识到这种肿瘤很重要。