İstanbul University, İstanbul Faculty of Medicine, Department of Dermatology and Venereology, İstanbul, Turkey
İstanbul University, İstanbul Faculty of Medicine, Department of Pathology, İstanbul, Turkey
Turk J Haematol. 2021 Feb 25;38(1):49-56. doi: 10.4274/tjh.galenos.2020.2020.0685. Epub 2021 Jan 4.
Lymphomatoid papulosis (LyP) is an indolent skin disease with variable clinical features classified among the primary cutaneous CD30+ T-cell lymphoproliferative disorders. It may show association with cutaneous and systemic lymphomas. We aimed to identify the frequency and characteristics of associated lymphomas among Turkish patients with LyP and to determine the risk factors for secondary lymphomas.
The files of patients diagnosed with LyP between 1998 and 2018 in a tertiary dermatology clinic were retrospectively analyzed. Univariate and multivariate models were used to assess the possible risk factors for secondary lymphomas, such as demographic and clinical characteristics of the patients.
Among 61 patients (47 adults, 14 children) with LyP, a total of 22 secondary lymphomas were observed in 20 patients. Nineteen of them were adults. Mycosis fungoides (MF) was the major associated lymphoma (n=19) followed by systemic anaplastic large cell lymphoma (ALCL) (n=2) and primary cutaneous ALCL (n=1). The most common stage in patients with accompanying MF was stage IB (n=11). While 18 patients showed the classical type of MF, one patient had folliculotropic MF. When the risk factors for association between LyP and other lymphomas were evaluated, only older age was found to be a significant risk factor and existence of ulcerated lesions was found to be a negative indicator.
LyP is not rare in the pediatric population. MF is the most common associated lymphoma in patients with LyP. Adult LyP patients are more commonly associated with secondary lymphomas than pediatric patients. Older age at the time of diagnosis of LyP is a significant risk factor for associated lymphomas.
蕈样肉芽肿（LyP）是一种惰性皮肤病，具有不同的临床特征，属于原发性皮肤 CD30+T 细胞淋巴增生性疾病。它可能与皮肤和系统性淋巴瘤有关。我们旨在确定土耳其 LyP 患者中相关淋巴瘤的频率和特征，并确定继发淋巴瘤的危险因素。
回顾性分析了 1998 年至 2018 年间在一家三级皮肤科诊所诊断为 LyP 的患者的档案。使用单变量和多变量模型评估了继发淋巴瘤的可能危险因素，如患者的人口统计学和临床特征。
在 61 例 LyP 患者（47 例成人，14 例儿童）中，共有 20 例患者的 22 例继发淋巴瘤。其中 19 例为成人。蕈样真菌病（MF）是主要的相关淋巴瘤（n=19），其次是系统性间变性大细胞淋巴瘤（ALCL）（n=2）和原发性皮肤 ALCL（n=1）。伴有 MF 的患者最常见的分期为 IB 期（n=11）。18 例患者表现为经典型 MF，1 例患者表现为滤泡性 MF。当评估 LyP 与其他淋巴瘤之间关联的危险因素时，只有年龄较大被发现是一个显著的危险因素，而存在溃疡性病变被发现是一个负性指标。
LyP 在儿科人群中并不罕见。MF 是 LyP 患者最常见的相关淋巴瘤。与儿科患者相比，成年 LyP 患者更常与继发性淋巴瘤相关。LyP 诊断时年龄较大是相关淋巴瘤的一个显著危险因素。
