蕈样肉芽肿病：180 例患者研究中的治疗反应和相关淋巴瘤。
Lymphomatoid papulosis: Treatment response and associated lymphomas in a study of 180 patients.
机构信息
Department of Dermatology, The University of Texas, MD Anderson Cancer Center, Houston, Texas; Department of Dermatology, Paracelsus Medical University, Salzburg, Austria.
Department of Dermatology, The University of Texas, MD Anderson Cancer Center, Houston, Texas.
出版信息
J Am Acad Dermatol. 2016 Jan;74(1):59-67. doi: 10.1016/j.jaad.2015.09.013. Epub 2015 Oct 28.
BACKGROUND
Lymphomatoid papulosis (LyP) is a CD30(+) lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology.
OBJECTIVE
The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center.
METHODS
Patient charts and clinical and histopathologic data of 180 patients with LyP were retrospectively assessed.
RESULTS
A total of 56.7% of patients was men. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6%, and mixed subtype in 4.4% of the patients. One hundred fourteen lymphomas were observed in 93 patients, with mycosis fungoides (61.4%) and anaplastic large cell lymphoma (26.3%) being the most common forms. Risk factors for development of lymphoma included sex and histologic subtype. Number of lesions and symptom severity were not associated with lymphoma development. Patients with type D were less likely to have lymphomas. Treatment provided symptomatic relief but did not prevent progression to lymphoma.
LIMITATIONS
The limitation of this study is the retrospective study design.
CONCLUSION
Patients with LyP are at increased risk of associated lymphomas. Thorough patient counseling is needed and long follow-up periods are required to detect and treat secondary lymphomas.
背景
蕈样肉芽肿（LyP）是一种 CD30(+) 的淋巴增生性疾病，具有自限性临床病程和恶性组织病理学特征。
目的
本研究旨在评估 MD 安德森癌症中心大型队列中 LyP 的特征、风险因素、相关恶性肿瘤、长期结局和治疗。
方法
回顾性评估了 180 例 LyP 患者的病历和临床及组织病理学数据。
结果
患者中 56.7%为男性。组织学亚型 A 占 47.2%，亚型 B 占 17.2%，亚型 C 占 22.8%，亚型 D 占 7.8%，亚型 E 占 0.6%，混合亚型占 4.4%。93 例患者中有 114 例淋巴瘤，蕈样真菌病（61.4%）和间变大细胞淋巴瘤（26.3%）是最常见的类型。发生淋巴瘤的危险因素包括性别和组织学亚型。病变数量和症状严重程度与淋巴瘤的发生无关。D 型患者发生淋巴瘤的可能性较小。治疗可缓解症状，但不能预防淋巴瘤的进展。
局限性
本研究的局限性在于回顾性研究设计。
结论
LyP 患者发生相关淋巴瘤的风险增加。需要对患者进行彻底的咨询，并需要进行长期随访以检测和治疗继发性淋巴瘤。
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