Evaluation of Associated Lymphomas and Their Risk Factors in Patients with Lymphomatoid Papulosis: A Retrospective Single- Center Study from Turkey

Objective: 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. Materials and Methods: 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. Results: 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. Conclusion: 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.

Lenfomatoid Papülozlu Hastalarda Eşlik Eden Lenfomaların ve Risk Faktörlerinin Değerlendirilmesi: Retrospektif Tek Merkezli Bir Çalışma

Amaç: Lenfomatoid papüloz (LeP), primer kutanöz CD30+ T-hücreli lenfoproliferatif hastalıklar arasında sınıflandırılan, değişken klinik özellikler gösterebilen bir deri hastalığıdır. Kutanöz ve sistemik lenfomalarla birliktelik gösterebilir. Bu çalışmada LeP’li Türk hastalarda ikincil lenfomaların sıklığını, özelliklerini ve gelişimleri açısından risk faktörlerini belirlemeyi hedefledik. Gereç ve Yöntemler: Üçüncü basamak bir dermatoloji kliniğinde 1998-2018 yılları arasında LeP tanısı alan hastaların dosyaları retrospektif olarak incelendi. Hastaların demografik ve klinik özellikleri gibi ikincil lenfomalar açısından risk oluşturabilecek faktörler tek değişkenli ve çok değişkenli istatistik modeller kullanılarak araştırıldı. Bulgular: LeP’li 61 hastanın (47 yetişkin, 14 çocuk) 20’sinde toplam 22 ikincil lenfoma görüldü. Bu hastalardan 19’u yetişkindi. Mikozis fungoides (MF) en sık görülen ikincil lenfomaydı (n=19) ve onu sistemik anaplastik büyük hücreli lenfoma (ABHL) (n=2) ile primer kutanöz ABHL (n=1) takip ediyordu. Eşlik eden MF hastalarında evre en sık IB (n=11) olarak bulundu. Onsekiz hastada klasik tip MF varken, bir hastada bu kutanöz lenfomanın folikülotropik tipi gözlendi. LeP ve ikincil lenfomalar arasındaki ilişki değerlendirildiğinde, sadece “ileri yaş” anlamlı bir risk faktörü olarak bulundu. “Ülsere lezyon varlığı” ikincil bir lenfoma bulunması için negatif bir gösterge olarak değerlendirildi. Sonuç: LeP pediatrik popülasyonda nadir değildir. LeP’ye en sık eşlik eden ikincil lenfoma MF’dir. İkincil lenfomalar erişkin LeP hastalarında çocuk hastalara göre daha sık görülür. LeP tanısı sırasında yaşın büyük olması ikincil bir lenfoma için anlamlı bir risk faktörüdür.

Kaynakça

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Kaynak Göster

Turkish Journal of Hematology
  • ISSN: 1300-7777
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 1999

350

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