Marrero-Alemán Gabriel, Montenegro-Dámaso Társila, Peñate Yeray
*Department of Dermatology, Complejo Hospitalario Universitario Insular Materno-Infantil, Universidad de Las Palmas de Gran Canaria, Canary Islands, Spain; and †Department of Pathology, Complejo Hospitalario Universitario Insular Materno-Infantil. Canary Islands, Spain.
Am J Dermatopathol. 2017 Oct;39(10):785-787. doi: 10.1097/DAD.0000000000000874.
Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) represents approximately 20% of cutaneous B lymphomas with an intermediate prognosis. Spontaneous regression is uncommon; there are only 2 published cases. An 83-year-old woman presented 2 orange erythematous nodules on the back of her right leg with an elastic consistency, infiltrated, painful to the touch, and of an 8-month evolution. A histological examination revealed a dense cellular dermo-hypodermic infiltrate sparing the papillary dermis, composed of large cells with immunoblast and centroblast morphology and frequent mitosis. Immunohistochemical studies showed positivity for CD20, CD79, Bcl2, Bcl6, MUM1, Fox-P1, and IgM with Ki67 >95%. Rearrangement of heavy IgH chains was monoclonal. The extension study was negative, establishing a diagnosis of PCDLBCL-LT, T2aN0M0. Three months after biopsy, the patient's lesions regressed spontaneously. New biopsies were taken that revealed a mild diffused dermo-hypodermic cellular infiltrate compounded by small-sized T lymphocytes, with predominance of CD8. Despite its self-limited character, treatment with radiotherapy was done, remaining asymptomatic after 1 year follow-up. There are 2 published cases of PCDLBCL-LT with spontaneous regression. The cause of this unusual autoinvolutional phenomenon is unknown; it may be an immune response against tumor cells through a traumatic or infectious mechanism.
原发性皮肤弥漫性大B细胞淋巴瘤,腿部型(PCDLBCL-LT)约占皮肤B淋巴瘤的20%,预后中等。自发消退并不常见,仅有2例报道。一名83岁女性,右小腿后部出现2个橙色红斑结节,质地有弹性,有浸润,触痛,病程8个月。组织学检查显示致密的细胞性真皮-皮下浸润,不累及乳头真皮,由具有免疫母细胞和中心母细胞形态且有频繁核分裂的大细胞组成。免疫组化研究显示CD20、CD79、Bcl2、Bcl6、MUM1、Fox-P1和IgM呈阳性,Ki67>95%。重链IgH基因重排为单克隆性。扩展检查为阴性,确诊为PCDLBCL-LT,T2aN0M0。活检后3个月,患者病变自发消退。再次活检显示轻度弥漫性真皮-皮下细胞浸润,伴有小尺寸T淋巴细胞,以CD8为主。尽管具有自限性,但仍进行了放射治疗,随访1年后无症状。已有2例PCDLBCL-LT自发消退的报道。这种不寻常的自 involutional现象的原因尚不清楚;可能是通过创伤或感染机制对肿瘤细胞的免疫反应。 (注:原文中“autoinvolutional”疑似拼写错误,可能是“auto-involutional”,翻译时按推测的正确形式翻译为“自 involutional”,请根据实际情况确认)