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Sézary综合征的治疗

Authors
Ellen J Kim, MD
Alain H Rook, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD
Translators
刘微, 主治医师

引言

Sézary综合征(Sézary syndrome, SS)是一种罕见的、皮肤T细胞淋巴瘤(cutaneous T cell lymphoma, CTCL)侵袭性亚型。SS和蕈样肉芽肿(mycosis fungoides, MF)密切相关但有所不同。SS表现为剥脱性红皮病以及有显著数量的循环恶性T细胞(Sézary细胞)。在一些患者中，SS可以累及淋巴结和内脏器官。

SS要么被认为是从MF演变而来(即，发病最初不符合SS的标准)或者是可能从开始即具有SS的全部症状和体征。不论是哪种情况，MF和SS都是根据同样的组织学标准来进行定义和分期。自2007年以后，使用与MF相同的TNMB分类系统，SS已被定义为T4(红皮病)皮肤表现伴B2(如，Sézary细胞绝对计数至少为1000个/uL)血液受累(表 1A)。与斑片/斑块型MF对比，SS通常症状更为明显，并且缓解可能性和预期生存率均更低。

SS的治疗将讨论在此。SS的临床表现、诊断和分期，以及MF的诊断和治疗，参见其他专题。 (参见“Clinical presentation, pathologic features, and diagnosis of Sézary syndrome”和“蕈样肉芽肿和Sézary综合征的分期和预后”和“蕈样肉芽肿的临床表现、病理特征和诊断”和“早期(ⅠA期至ⅡA期)蕈样肉芽肿的治疗”和“进展期(ⅡB-Ⅳ期)蕈样肉芽肿的治疗”)

分期

MF和SS的标准分期系统是基于对皮肤(T)、淋巴结(N)、内脏受累(M)和血液(B)的评估。详细内容参见其他专题(表 1A和表 1B)。 (参见“蕈样肉芽肿和Sézary综合征的分期和预后”，关于‘分期’一节)

SS相当于MF的TNMB分类系统中T4加B2。T4红皮病性皮肤病描述了红斑受累不低于80%体表面积(body surface area, BSA)的病例的特征。B2受累反映确定了T细胞克隆性增殖，其中Sézary细胞绝对计数通常至少为1000个/uL。 (参见“Clinical presentation, pathologic features, and diagnosis of Sézary syndrome”, section on ‘Diagnostic criteria’)

                                     

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Literature review current through: 2017-06 . | This topic last updated: 2017-02-08.
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