皮肤淋巴瘤

蕈样霉菌病(MF)
蕈样霉菌病是一种常见嗜表皮性的皮肤T细胞淋巴瘤 （CTCL），特点是增生的小至中等大小的T淋巴细胞， 有“脑回样”的核。MF是最常见的皮肤T细胞淋巴瘤， 约占所有皮肤原发性淋巴瘤的50%。
Clinical features MF has an indolent clinical course with slow progression over years or sometimes decades, from patches to more infiltrated plaques and eventually to tumors .
The prognosis of patients is dependent on stage. In recent studies, 10-year disease-specific survivals were 97-98% for patients with limited patch/plaque disease, 83% for patients with generalized patch/plaque disease, 42% for patients with tumor stage disease.
遗传学特征：
克隆性的T细胞受体基因重排见于多数病例。许多 染色体的结构和数目的异常已有描述，尤其是进展 期的MF，但恒定的MF特异的染色体异位还未发现。 10号染色体长臂的缺失和P15、P16、P53肿瘤抑制 基因的异常可见于多数的MF患者。
Prognosis and predictive factors.
原发性皮肤间变性大细胞淋巴瘤
Primary cutaneous lymphomas are
currently classified by the European Organization for Research and Treatment of Cancer (EORTC) classification or the World Health Organization (WHO) classification, but both systems have shortcomings. During recent consensus meetings representatives of both systems reached agreement on a new classification, which is now called the WHO-EORTC classification.
组织病理学：
SS最主要的特征与MF的特征类似。然而SS 中的浸润细胞成分更单一，可无“嗜表皮现 象”。累及淋巴结时可特征性地出现出弥漫、 单一的Sézary细胞浸润，伴有淋巴结正常结 构的破坏。 免疫表型： 肿瘤性的T细胞呈CD3+、CD4+、CD8－的 表型。血循环中的Sézary细胞常表现出CD7 和CD26的丢失。
Immunophenotype
The neoplastic cells in MF have a mature CD3, CD4, CD45RO, CD8 memory T-cell phenotype. Demonstration of an aberrant phenotype (loss of pan–T-cell antigens such as CD2, CD3, and CD5) is often seen and is an important adjunct in the diagnosis of MF.
遗传学特征： T细胞受体基因呈现克隆性的重排。在周围血中 出现克隆性的T细胞是重要的诊断依据。特定的 染色体异常尚未在SS中发现，但复杂的核型常 见。有研究证实在SS中的一个与MF的几乎相同 的特征性的染色体结构异常。提示这两类疾病代 表同一疾病谱不同部分、有相似的病因。
Prognosis and predictive factors.
预后及预测因素： MF患者的预后取决于分期。局限性的斑片/斑 块期的MF患者，10年生存率是97~98%；弥 漫的斑片/斑块期的MF患者是83%；肿块期的 是42%。
Sézary syndrome (SS)
Sézary syndrome is defined historically by the triad of erythroderma, generalized lmphadenopathy, and the presence of neoplastic T cells (Se´zary cells) in skin, lymph nodes, and peripheral blood.
Primary cutaneous anaplastic large-cell Lymphoma (CALCL)
CALCL is composed of large cells with an anaplastic, pleomorphic, or immunoblastic cytomorphology and expression of the CD30 antigen by the majority (more than 75%) of tumor cells. There is no clinical evidence or history of LyP, MF, or another type of CTCL.
Genetwenku.baidu.comc features.
T-cell receptor genes are clonally rearranged. Recurrent chromosomal translocations have not been detected in SS, but complex karyotypes are common. Several studies have identified a consistent pattern of identical chromosomal abnormalities in SS, which was almost identical to that in MF, suggesting that both conditions represent parts of the same spectrum of disease with a similar pathogenesis。
The prognosis is generally poor, with a median survival between 2 and 4 years, depending on the exact definition used. Most patients die of opportunistic infections that are due to immunosuppression.
免疫表型： MF中的肿瘤细胞有成熟型的CD3+、CD4+、 CD45RO+、CD8－的T记忆细胞的免疫表型。 出现奇异的表型（如丢失全T细胞抗原CD2、 CD3、CD5等）常见，可作为诊断MF的参考 指标。
Genetic features.
Clonal T-cell receptor gene rearrangements are detected. Many structural and numerical chromosomal abnormalities have been described, but MF-specific chromosomal translocations have not been identified.
预后及预测因素： 预后通常不佳，中位生存时间约2~4年，与诊断 界定有关。多数病人死于由免疫抑制引起的机会 性感染。
原发性皮肤CD30阳性的淋巴增殖性 疾病（LPD）
LPD是第二种最常见的皮肤T细胞淋巴瘤，大
约占CTCL的30%。这组中包括原发性皮肤间 变性大细胞淋巴瘤（C-ALCL）、淋巴瘤样丘 疹病（LyP）和交界病例。现在普遍认为CALCL和LyP形成一种疾病的谱系。 “交界性 病变” 是指那些尽管仔细分析临床病理的相 关性仍不能准确区分C-ALCL和LyP的病例。 通过临床进一步随访检查病人将最终确诊病 人为C-ALCL还是LyP。
Sézary综合症 (SS)
Sézary综合症包括了既往的“红皮病”、泛发的 淋巴结病和在皮肤、淋巴结及血液中出现肿瘤性 T细胞（Sézary细胞）的三联症。
Clinical features
SS is a rare disease and occurs exclusively in adults. It is characterized by erythroderma, which may be associated with marked exfoliation, edema, and lichenification, and which is intensely pruritic. Lymphadenopathy, alopecia, onychodystrophy,and palmoplantar hyperkeratosis are common findings. 临床特征： SS是一种罕见病，毫无例外地发生在成人。特征性地 表现为红皮病，可相关性地出现明显褪皮、水肿和“苔 藓样”硬化，这会造成奇痒。淋巴结病、秃头、甲床萎 缩和掌柘部位角化亢进是常见的症状。
Mycosis Fungoides (MF)
MF is a commonly epidermotropic CTCL characterized by a proliferation of small- to mediumsized T lymphocytes with cerebriform nuclei.
临床特征：
MF多发在成人，有惰性的临床病程、进展缓慢，从 斑块到浸润的斑块最后到肿瘤经时数年，有时可达 十几年。在部分病人，淋巴结和内脏器官可能会在 疾病的晚期被累及。肿块期的MF病人可特征性地同 时出现斑点、斑块和瘤块，后者多出现溃疡。
组织病理学： MF的早期斑块状的病损表现为表浅的“条带样”或 “苔藓样”浸润，主要成份是淋巴细胞和组织细胞。 异型的瘤细胞数量不多，细胞小至中等大小、有 “脑回状”的核，多数局限在表皮（嗜表皮性）。 肿瘤细胞特征性地沿表皮的基底层生长，呈“晕环 状”或“线状”的排列。出现表皮间的异型淋巴细 胞的聚集（Pautrier微脓肿）是一种特征性的特点， 但仅可见于一小部分病例。
WHO-EORTC classification for cutaneous lymphomas
汇报者：权晟
introduction
The term “primary cutaneous lymphoma” refers
to cutaneous T-cell lymphomas (CTCLs) and cutaneous B-cell lymphomas (CBCLs) that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. Primary cutaneous lymphomas often have a completely different clinical behavior and prognosis from histologically similar systemic lymphomas, which may involve the skin secondarily, and therefore require different types of treatment.