Raychaudhuri Sreejata, Rahvar Maral, Jedrych Jaroslaw, Karunamurthy Arivarasan, Kruglov Oleg, Rakfal Susan, Kane Kevin, Akilov Oleg E
Department of Internal Medicine, University of Pittsburgh Medical Center, McKeesport, PA.
Department of Dermatology, University of Pittsburgh, Pittsburgh, PA.
Am J Dermatopathol. 2018 Nov;40(11):836-840. doi: 10.1097/DAD.0000000000001188.
Cutaneous peripheral T-cell lymphoma, not otherwise specified represents a "waste basket" of all cases that cannot be put into another of the categories of mature cutaneous T-cell lymphoma. Previously, the sudden multifocal development of cutaneous CD4 tumors without preceding a patch or plaque stage was classified as d'emblée form of mycosis fungoides (MF). Currently, the term "MF" reserved only for the classic Alibert-Bazin type characterized by the evolution of patches, plaques, and tumors or for variants showing a similar clinical course. The authors describe a 75-year-old white woman who presented with a solitary skin tumor in the right supraclavicular region, with no lymph node or systemic involvement. Local external beam radiation treatment resulted in a complete response. The patient relapsed after 5 months with new tumors in the left neck and left upper chest. Biopsy of the lesions showed a dermal infiltrate of atypical small- to medium-sized T-lymphocytes, and immunohistochemical staining showed coexpression of CD4/CD8 in a subset of these cells, which was confirmed with flow cytometry of the tumor. Although the patient had no preceding patch or plaque stage, the authors herein report this extremely rare case of CD4/CD8 dual-positive peripheral T-cell lymphoma, not otherwise specified presented as MF d'emblée and discuss the seldom similar cases published previously.
皮肤外周T细胞淋巴瘤，非特殊类型代表了所有无法归入其他成熟皮肤T细胞淋巴瘤类别的病例的“垃圾桶”。以前，皮肤CD4肿瘤在无前驱斑片或斑块期的情况下突然多灶性发展被归类为蕈样肉芽肿（MF）的即刻型。目前，“MF”一词仅保留用于以斑片、斑块和肿瘤演变或显示类似临床病程的变体为特征的经典阿利贝尔 - 巴赞型。作者描述了一名75岁的白人女性，她在右锁骨上区域出现一个孤立性皮肤肿瘤，无淋巴结或全身受累。局部外照射放疗导致完全缓解。患者在5个月后复发，左颈部和左上胸部出现新肿瘤。病变活检显示真皮内有非典型小至中等大小T淋巴细胞浸润，免疫组化染色显示这些细胞亚群中CD4/CD8共表达，肿瘤的流式细胞术证实了这一点。尽管该患者无前驱斑片或斑块期，但作者在此报告了这例极为罕见的CD4/CD8双阳性外周T细胞淋巴瘤，非特殊类型，表现为MF即刻型，并讨论了先前发表的极少类似病例。
