Department of Pathology, Hôpital Necker-Enfants-Malades (AP-HP), Université Paris-Descartes, Paris, France.
Dermatology. 2010;220(3):226-33. doi: 10.1159/000269737. Epub 2010 Feb 10.
Richter's syndrome (RS) corresponds to the transformation of chronic lymphocytic leukemia (CLL) into high-grade lymphoma. RS can involve extranodal sites including the gastrointestinal tract, lungs and skin. Cutaneous RS is rare, we describe 4 cases with clinical manifestations, histological and immunohistological patterns, and outcome.
Clinical data were analyzed and all patients' skin biopsy samples stained with HE for the CD20, CD5, CD3 and CD30 antigens. Epstein-Barr-virus (EBV)-encoded early RNA and clonal rearrangements were also analyzed.
The patients' mean age at CLL diagnosis was 57 years (53-62 years), with a male/female sex ratio of 3:1. The transformation to cutaneous RS occurred between 8 and 75 months after initial diagnosis and progressed to a fatal systemic disease in 3 cases, between 24 and 129 months. Cutaneous CLL was associated with earlier transformation in our series and could not be distinguished from RS on clinical grounds alone. All patients had a large-cell infiltrate and clonal rearrangements.
The precise mechanism of RS is unclear, but a role of EBV has been suggested in fludarabine-treated CLL. For all our patients, the diagnosis of transformation was made on the basis of cutaneous localizations and led to intensified CLL treatment.
里希特综合征（RS）对应于慢性淋巴细胞白血病（CLL）向高级别淋巴瘤的转化。RS 可累及包括胃肠道、肺和皮肤在内的结外部位。皮肤 RS 较为罕见，我们描述了 4 例具有临床表现、组织学和免疫组织化学模式以及结局的病例。
分析了临床数据，对所有患者的皮肤活检样本进行了 CD20、CD5、CD3 和 CD30 抗原的 HE 染色。还分析了 EBV 编码的早期 RNA 和克隆重排。
患者在 CLL 诊断时的平均年龄为 57 岁（53-62 岁），男女比例为 3:1。从初始诊断到皮肤 RS 转化的时间间隔为 8 至 75 个月，其中 3 例在 24 至 129 个月时进展为致命性系统性疾病。在本系列中，皮肤 CLL 与更早的转化相关，仅凭临床依据无法将其与 RS 区分开来。所有患者均有大细胞浸润和克隆重排。
RS 的确切机制尚不清楚，但 EBV 的作用已在氟达拉滨治疗的 CLL 中得到提示。对于我们所有的患者，转化的诊断是基于皮肤定位，并导致 CLL 治疗的强化。
