Burg G, Häffner A, Böni R, Dommann S, Dummer R
Department of Dermatology, University of Zurich Medical School, Switzerland.
Recent Results Cancer Res. 1995;139:225-37. doi: 10.1007/978-3-642-78771-3_17.
Cutaneous T cell lymphomas (CTCL) are lymphoproliferative disorders, which can be classified by an adaptation of the Kiel classification. The most common CTCL, Mycosis fungoides (MF) and Sézary syndrome (SS), are monoclonal T helper memory lymphomas. They belong to the group of peripheral T cell lymphomas. However, CTCL includes other disease entities such as granulomatous slack skin, pagetoid reticulosis, and lipotropic CTCL. Polymerase chain reaction of the T cell receptor (TCR)-gamma and screening of the PCR products for sequence-specific mobility in acrylamide gels has increased the detection limit for clonal T cells in the skin. This method will help to define the relationship of CTCL to other lymphoproliferative disorders and may contribute to the early diagnosis of CTCL. In SS, the malignant CTCL clone secretes a T helper-2 cytokine pattern, which might be responsible for the systemic immunosuppression in CTCL patients. New treatment modalities (IL-12) might correct these immunologic abnormalities. Cutaneous B cell lymphomas mostly are follicular center cell derived and usually have a good prognosis. Therapy of cutaneous lymphomas should take into account prognostic factors, the most reliable of which in CTCL is the tumor mass expressed by the tumor burden index.
皮肤T细胞淋巴瘤（CTCL）是淋巴增殖性疾病，可通过改编的基尔分类法进行分类。最常见的CTCL，蕈样肉芽肿（MF）和 Sézary 综合征（SS），是单克隆T辅助记忆淋巴瘤。它们属于外周T细胞淋巴瘤组。然而，CTCL还包括其他疾病实体，如肉芽肿性皮肤松弛症、派杰样网状细胞增生症和亲脂性CTCL。T细胞受体（TCR）-γ的聚合酶链反应以及对PCR产物在丙烯酰胺凝胶中的序列特异性迁移率进行筛选，提高了皮肤中克隆性T细胞的检测限。该方法将有助于确定CTCL与其他淋巴增殖性疾病的关系，并可能有助于CTCL的早期诊断。在SS中，恶性CTCL克隆分泌T辅助2细胞因子模式，这可能是CTCL患者全身免疫抑制的原因。新的治疗方式（IL-12）可能会纠正这些免疫异常。皮肤B细胞淋巴瘤大多起源于滤泡中心细胞，通常预后良好。皮肤淋巴瘤的治疗应考虑预后因素，在CTCL中最可靠的预后因素是由肿瘤负荷指数表示的肿瘤肿块。
