Department of Dermatology, University Hospital Zürich, Zürich, Switzerland.
G Ital Dermatol Venereol. 2012 Dec;147(6):553-62.
Cutaneous peripheral T-cell lymphoma, unspecified/not otherwise specified (PTL NOS) represents a phenotypically and prognostically heterogenous group of cutaneous T-cell lymphomas (CTCL) that do not fit into any of well defined defined CTCL subtypes. In the WHO-EORTC classification as well as the WHO classification, three entities have been delineated as provisional rare subtypes of PTL based on their characteristic clinico-pathological, immunophenotypic and prognostic features and have been separated out from PTL, NOS: Primary cutaneous CD4-positive small/medium T-cell lymphoma (CD4+ SMTL), primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma (CD8+ AECTCL), and primary cutaneous gamma/delta T-cell lymphoma (CGD-TCL). CD4+ SMTL manifests in most patients with a solitary nodule in the head and neck area and nodular infiltrates of CD4+ small to medium-sized lymphocytes with nuclear pleomorphism. The prognosis of this lymphoproliferation is excellent in patients with a solitary lesion, but may be impaired in patients with multifocal disease. Rapidly evolving erosive or necrotic plaques and nodules with an epidermotropic infiltrate of CD8+ atypical lymphocytes are the hallmark of CD8+ AECTCL, which exhibits a poor prognosis. CGD-TCL displays a broad spectrum of clinical and histological manifestations with expression of the T-cell receptor gamma/delta chain as the common denominator and diagnostic marker. As most of other forms of PTL, CGD-TCL carries a poor prognosis. Despite the rarity of PTL NOS, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CTC, especially since most of these lymphomas exhibit an unfavourable prognosis. Immediate intense treatment with multiagent chemotherapy and hematopoietic stem cell transplantation is indicated in patients with PTL NOS. This review focuses on the clinicopathological aspects, the diagnostic criteria and the classification of the rare subtypes of PTL and PTL NOS.
未特指/不能分类的皮肤外周 T 细胞淋巴瘤（PTL NOS）代表了一组表型和预后异质性的皮肤 T 细胞淋巴瘤（CTCL），它们不符合任何明确定义的 CTCL 亚型。在世界卫生组织-欧洲癌症研究与治疗组织（WHO-EORTC）分类和世界卫生组织（WHO）分类中，根据其特征性临床病理、免疫表型和预后特征，已经确定了三种实体作为 PTL 的暂定罕见亚型，并从 PTL，NOS 中分离出来：原发性皮肤 CD4 阳性小/中 T 细胞淋巴瘤（CD4+ SMTL）、原发性皮肤 CD8 阳性侵袭性表皮 T 细胞淋巴瘤（CD8+ AECTCL）和原发性皮肤γ/δ T 细胞淋巴瘤（CGD-TCL）。CD4+ SMTL 大多数患者表现为头颈部孤立性结节和 CD4+小至中等大小淋巴细胞的结节性浸润，具有核多形性。对于单发病变的患者，这种淋巴细胞增生的预后极好，但对于多发病灶的患者，预后可能会受损。快速进展的侵蚀性或坏死性斑块和结节，伴有 CD8+异型淋巴细胞的表皮浸润，是 CD8+ AECTCL 的标志，其预后不良。CGD-TCL 具有广泛的临床表现和组织学表现，以 T 细胞受体γ/δ链的表达为共同特征和诊断标志物。与其他形式的 PTL 一样，CGD-TCL 的预后较差。尽管 PTL NOS 较为罕见，但临床医生以及皮肤科医生和病理学家都应该熟悉这些罕见的 CTC，尤其是因为大多数这些淋巴瘤的预后不佳。对于 PTL NOS 患者，应立即采用多药化疗和造血干细胞移植进行强烈治疗。本文重点介绍了罕见 PTL 亚型和 PTL NOS 的临床病理特征、诊断标准和分类。
