1 Division of Dermatology, University of Toronto, Toronto, ON, Canada.
J Cutan Med Surg. 2017 Nov/Dec;21(6):507-512. doi: 10.1177/1203475417716366. Epub 2017 Jun 14.
Lymphomatoid papulosis is one of the primary cutaneous CD30+ T-cell lymphoproliferative disorders. Although considered a benign disease, lymphomatoid papulosis has been associated potentially with an increased risk of secondary hematolymphoid malignancies.
The aim of this study was to assess the clinical characteristics and histologic subtypes of lymphomatoid papulosis, identify the prevalence and types of secondary hematolymphoid malignancies, and determine the potential risk factors for development of these hematolymphoid malignancies.
A retrospective chart review was performed for all histologically confirmed cases of lymphomatoid papulosis between 1991 and 2016.
Seventy patients with lymphomatoid papulosis were identified. Thirty patients (43%) experienced a secondary hematolymphoid malignancy. Twenty-four (80%) of the hematolymphoid malignancies occurred after the onset of lymphomatoid papulosis. Older age at diagnosis of lymphomatoid papulosis, male sex, histology type B, and the presence of T-cell receptor gene rearrangement are associated with higher risk of developing hematolymphoid malignancy.
Lymphomatoid papulosis is associated with increased risk of developing secondary hematolymphoid malignancies, particularly mycosis fungoides and cutaneous anaplastic large cell lymphoma.
蕈样肉芽肿是原发性皮肤 CD30+T 细胞淋巴增生性疾病之一。尽管蕈样肉芽肿被认为是一种良性疾病，但它与继发性血液淋巴恶性肿瘤的风险增加有关。
本研究旨在评估蕈样肉芽肿的临床特征和组织学亚型，确定继发性血液淋巴恶性肿瘤的患病率和类型，并确定发生这些血液淋巴恶性肿瘤的潜在危险因素。
对 1991 年至 2016 年间所有经组织学证实的蕈样肉芽肿病例进行回顾性图表审查。
共发现 70 例蕈样肉芽肿患者。30 例（43%）患者发生继发性血液淋巴恶性肿瘤。24 例（80%）血液淋巴恶性肿瘤发生在蕈样肉芽肿发病后。蕈样肉芽肿诊断时年龄较大、男性、组织学类型 B 以及 T 细胞受体基因重排与发生血液淋巴恶性肿瘤的风险增加相关。
蕈样肉芽肿与继发性血液淋巴恶性肿瘤（尤其是蕈样真菌病和皮肤间变性大细胞淋巴瘤）的风险增加相关。
