Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz

ATİLLA, Erden; AKAY, Bengü Nisa; Sanli, Hatice; Okcu Heper, Aylin; BOTSALI, Ayşenur; PARLAK, Nehir; Vural, Secil

Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz

Seçil VURAL, (Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey)

Bengü Nisa AKAY, (Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey)

Ayşenur BOTSALI, (Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey)

Erden ATİLLA, (Ankara University Faculty of Medicine, Department of Hematology, Ankara, Turkey)

Nehir PARLAK, (Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey)

Aylin Okçu HEPER, (Ankara University Faculty of Medicine, Department of Pathology, Ankara, Turkey)

Hatice ŞANLI (Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey)

Turkish Journal of Hematology

78 4

Yıl: 2018 Cilt: 35 Sayı: 1 Sayfa Aralığı: 35 - 41 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz

Öz:

Amaç: Transforme mikozis fungoides (T-MF) MF nadir görülen agresif seyirli bir alt tipidir. Bu çalışmada transformasyon gelişen MF/Sezary sendromu (SS) hastalarının klinik ve laboratuvar özelliklerinin değerlendirilmesi amaçlanmıştır.Gereç ve Yöntemler: Bu çalışmada tek bir referans merkezde 20002014 yılları arasında takip edilen MF/SS hastaları arasından T-MF geliştirenler retrospektif olarak değerlendirilmiştir. Demografik, klinik ve laboratuvar veriler, immünfenotiplendirme, tedavi yanıtları, histopatolojik özellikler ve sağkalım analiz edilmiştir.Bulgular: Takip edilen 254 MF hastası içerisinde 25 T-MF saptanarak (%10,2) çalışmaya dahil edilmiştir. Erkek kadın oranı 2,6/1'dir. MF tanısı ile T-MF tanısı arasında geçen sürenin medianı 32 ay olarak tespit edilmiştir . Dokuz hastada (%36) tanı anında transformasyon bulunmaktadır. İleri hastalık evresi ve yüksek serum laktat dehidrogenaz (LDH) düzeyleri kötü prognoz ve tedavi yanıtı göstergesi olarak saptanmıştır. Tedaviye dirençli 18 ileri evre hastadan beşine allojenik hematopoetik kök hücre transplantasyonu (allo-HKHT) yapılmıştır. Bunlardan üçünde tam remisyon sağlanmıştır. İzlemde toplam 10 hasta hastalık progresyonu nedeniyle kaybedilmiştir. T-MF sonrası ortalama sağkalım 25,2±14,9 aydır.Sonuç: İleri hastalık evresi, yüksek LDH düzeyi, perifer kan T hücrelerde CD26 ve CD7 kaybı kötü prognoz belirteçlerindendir. Tedaviye dirençli nodül ve tümörler T-MF açısından şüphe uyandırmalıdır. T-MF'de sağkalım kısalmıştır. Bazı hastalarda birinci basamak tedavilere iyi yanıt alınabilmektedir. Ancak birinci basamak tedavilere yanıtsız hastalar genellikle ikinci ve üçüncü basamak tedavilere de direnç gösterebilmektedir. Allo-HKHT, T-MF hastalarında alternatif bir tedavi yöntemi olarak kullanılabilir

Anahtar Kelime:

Konular: Hematoloji Biyoteknoloji ve Uygulamalı Mikrobiyoloji Onkoloji Enfeksiyon Hastalıkları Hücre Biyolojisi Tıbbi Laboratuar Teknolojisi

Transformation of Mycosis Fungoides/Sezary Syndrome: Clinical Characteristics and Prognosis

Öz:

Objective: Transformed mycosis fungoides (T-MF) is a rare variant of MF with an aggressive course. In this study, we aimed to describe characteristics of MF/Sezary syndrome (SS) patients with transformation.Materials and Methods: Patients diagnosed with T-MF among MF/SS patients between 2000 and 2014 in a tertiary single center were evaluated retrospectively. Demographic data, clinical data, laboratory data, immunophenotype features, response to treatment, survival, and histopathologic features were analyzed.Results: Among 254 MF patients, 25 patients with T-MF were identified (10.2%) and included in the study. The male-to-female ratio was 2.6/1. The median time between MF diagnosis and transformation was 32 months . Nine (36%) patients were diagnosed initially with T-MF. Advanced disease stage and high serum lactate dehydrogenase (LDH) levels were indicators of poor prognosis and treatment response. Five of the 18 patients with progressive disease had undergone allogeneic hematopoietic stem cell transplantation (allo-HSCT). Allo-HSCT resulted in complete remission in three (60%) patients. Ten (40%) patients died as a result of disease progression. Mean survival time was 25.2±14.9 months after transformation.Conclusion: Advanced stage, high serum LDH levels, and loss of CD26 and CD7 expression in the peripheral blood are poor rognostic factors in T-MF. Treatment-resistant tumors and nodules should be cautionary for T-MF. Patients with T-MF have a shortened survival. Some patients may respond to first-line treatments. However, the majority of patients who do not respond to first-line therapies also are unresponsive to second or third-line therapies. Allo-HSCT may be an alternative option in patients with T-MF

Anahtar Kelime:

Konular: Hematoloji Biyoteknoloji ve Uygulamalı Mikrobiyoloji Onkoloji Enfeksiyon Hastalıkları Hücre Biyolojisi Tıbbi Laboratuar Teknolojisi

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık

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APA	Vural S, AKAY B, BOTSALI A, ATİLLA E, PARLAK N, Okcu Heper A, Sanli H (2018). Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. , 35 - 41.
Chicago	Vural Secil,AKAY Bengü Nisa,BOTSALI Ayşenur,ATİLLA Erden,PARLAK Nehir,Okcu Heper Aylin,Sanli Hatice Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. (2018): 35 - 41.
MLA	Vural Secil,AKAY Bengü Nisa,BOTSALI Ayşenur,ATİLLA Erden,PARLAK Nehir,Okcu Heper Aylin,Sanli Hatice Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. , 2018, ss.35 - 41.
AMA	Vural S,AKAY B,BOTSALI A,ATİLLA E,PARLAK N,Okcu Heper A,Sanli H Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. . 2018; 35 - 41.
Vancouver	Vural S,AKAY B,BOTSALI A,ATİLLA E,PARLAK N,Okcu Heper A,Sanli H Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. . 2018; 35 - 41.
IEEE	Vural S,AKAY B,BOTSALI A,ATİLLA E,PARLAK N,Okcu Heper A,Sanli H "Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz." , ss.35 - 41, 2018.
ISNAD	Vural, Secil vd. "Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz". (2018), 35-41.

APA	Vural S, AKAY B, BOTSALI A, ATİLLA E, PARLAK N, Okcu Heper A, Sanli H (2018). Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. Turkish Journal of Hematology, 35(1), 35 - 41.
Chicago	Vural Secil,AKAY Bengü Nisa,BOTSALI Ayşenur,ATİLLA Erden,PARLAK Nehir,Okcu Heper Aylin,Sanli Hatice Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. Turkish Journal of Hematology 35, no.1 (2018): 35 - 41.
MLA	Vural Secil,AKAY Bengü Nisa,BOTSALI Ayşenur,ATİLLA Erden,PARLAK Nehir,Okcu Heper Aylin,Sanli Hatice Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. Turkish Journal of Hematology, vol.35, no.1, 2018, ss.35 - 41.
AMA	Vural S,AKAY B,BOTSALI A,ATİLLA E,PARLAK N,Okcu Heper A,Sanli H Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. Turkish Journal of Hematology. 2018; 35(1): 35 - 41.
Vancouver	Vural S,AKAY B,BOTSALI A,ATİLLA E,PARLAK N,Okcu Heper A,Sanli H Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz. Turkish Journal of Hematology. 2018; 35(1): 35 - 41.
IEEE	Vural S,AKAY B,BOTSALI A,ATİLLA E,PARLAK N,Okcu Heper A,Sanli H "Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz." Turkish Journal of Hematology, 35, ss.35 - 41, 2018.
ISNAD	Vural, Secil vd. "Mikozis Fungoides/Sezary Sendromunda Transformasyon: Klinik Özellikler ve Prognoz". Turkish Journal of Hematology 35/1 (2018), 35-41.