Winkler C F, Bunn P A
Crit Rev Oncol Hematol. 1983;1(1):49-92. doi: 10.1016/s1040-8428(83)80004-3.
Cutaneous T-cell lymphomas define a spectrum of disorders associated with T-lymphocytic proliferation with clinical manifestations occurring in the skin during the course of the disease. This review has dealt with two rather uncommon disorders, namely mycosis fungoides and Sezary syndrome which are indolent malignant lymphomas, occurring primarily in the fifth decade, and affecting males most frequently. Historically, mycosis fungoides and Sezary syndrome have been described for a relatively short time. As witnessed by Table 2, little was known concerning these disorders, other than clinical and pathologic features, until the application of immunologic, cell biologic, and cytogenetic technology which burgeoned a multitude of questions. The discovery of TCGF has allowed for both continuous growth of normal and neoplastic T cells and for the clonal expansion of some malignant clones. The establishment of these continuous cultures allowed for: (1) investigation of the mechanism of TCGF production and stimulation of T-cell growth, and (2) identification of HTLV, a retrovirus found in cell cultures from two patients with CTCL, and subsequently from patients with Japanese adult T-cell lymphoma. In addition, the HTLV has been related to a more virulent form of T-cell malignancy. The exact etiologic role of this virus in the CTCL is presently the subject of intense investigation. Through the use of immunologic methods the malignant cell of CTCL has been pheno-typically and functionally characterized as a "helper/inducer" subtype (E rosette+, anti-T-cell antisera+, T11+, T1+, T3+, 3A1-, T6-, T8-) and usually Ia-, HLADR-. Clinical manifestations of the phenotype may be clinically apparent in the serologic abnormalities present in these disorders. Utilizing these methods to investigate these disorders may provide a key to the understanding of T-cell function and cellular immunity much as myeloma provided a model for the understanding of B cells and immunity. Clinically and pathologically, these disorders behave as malignant indolent lymphomas with spread from localized cutaneous lesions to extracutaneous involvement of the blood, lymph nodes, and viscera culminating in the death of the patient from either organ dysfunction or infectious complications. At autopsy, this extracutaneous involvement is more pronounced than what was expected ante-mortem. Application of prospective staging techniques employing such special procedures as E-rosette cytology, cytogenetics, and electron microscopy in addition to usual light microscopy studies has demonstrated a greater percentage of extracutaneous involvement than otherwise expected.(ABSTRACT TRUNCATED AT 400 WORDS)
皮肤T细胞淋巴瘤是一组与T淋巴细胞增殖相关的疾病，在疾病过程中会出现皮肤临床表现。本综述探讨了两种相对罕见的疾病，即蕈样肉芽肿和塞扎里综合征，它们是惰性恶性淋巴瘤，主要发生在五十多岁，男性最常受累。从历史上看，蕈样肉芽肿和塞扎里综合征被描述的时间相对较短。如表2所示，在应用免疫、细胞生物学和细胞遗传学技术引发众多问题之前，除了临床和病理特征外，人们对这些疾病知之甚少。TCGF的发现使得正常和肿瘤性T细胞能够持续生长，一些恶性克隆也能克隆性扩增。这些连续培养物的建立使得：（1）能够研究TCGF产生和刺激T细胞生长的机制，以及（2）能够鉴定HTLV，一种在两名皮肤T细胞淋巴瘤患者的细胞培养物中发现的逆转录病毒，随后在日本成人T细胞淋巴瘤患者中也发现了该病毒。此外，HTLV与一种更具侵袭性的T细胞恶性肿瘤有关。这种病毒在皮肤T细胞淋巴瘤中的确切病因作用目前是深入研究的主题。通过使用免疫方法，皮肤T细胞淋巴瘤的恶性细胞在表型和功能上被特征化为“辅助/诱导”亚型（E玫瑰花结阳性、抗T细胞抗血清阳性、T11阳性、T1阳性、T3阳性、3A1阴性、T6阴性、T8阴性），通常Ia阴性、HLADR阴性。这些疾病表型的临床表现可能在这些疾病中存在的血清学异常中临床上明显可见。利用这些方法研究这些疾病可能为理解T细胞功能和细胞免疫提供关键，就像骨髓瘤为理解B细胞和免疫提供了模型一样。在临床和病理上，这些疾病表现为恶性惰性淋巴瘤，从局部皮肤病变扩散到血液、淋巴结和内脏的皮肤外受累，最终患者因器官功能障碍或感染并发症死亡。尸检时，这种皮肤外受累比生前预期的更为明显。除了常规的光学显微镜研究外，应用前瞻性分期技术，如E玫瑰花结细胞学、细胞遗传学和电子显微镜等特殊程序，已证明皮肤外受累的比例比预期的更高。（摘要截短至400字）
