Nörofibromatozis Tip1 Hastalarında Telomeraz Aktivitesi

Year 2018, Volume: 27 Issue: 2, 199 - 205, 30.06.2018

Serhan Küpeli

https://doi.org/10.17827/aktd.365700

Abstract

Çeşitli kanser türlerinde yapılan çalışmalarda kısa telomer uzunluğu ve düşük telomeraz aktivitesinin bazı kanser tiplerinde koruyucu etkisi olduğundan bahsedilmektedir. Nörofibromatozis Tip-1 (NF1) hastalarının bazı kanser türleri için yüksek risk altında olduğu bilinmektedir. Beyin tümörleri, malign periferik sinir kılıfı tümörleri, optik gliom, yumuşak doku sarkomları gibi malign tümörler ve nörofibromlar ve hamartomlar gibi benign olanlar bunlar arasında sayılabilir. İlginç olarak NF1 hastalarında ortaya çıkan kanserlerde prognoz genel olarak aynı tanılı NF1 olmayan hastalara göre daha iyi olmakta ve sağkalım daha uzun olmaktadır. Bu derlemede NF1 hastalarındaki telomeraz aktivitesinin gelişebilecek kanserlerle olan ilişkisi literatür bilgileri ışığında irdelenmiştir. NF1 hastalarında benign veya malign tümör gelişimi açısından telomeraz aktivitesi ölçümü tümör belirleyici işlevi görebilir.

Keywords

Nörofibromatozis Tip1, telomeraz aktivitesi

Telomerase Activity in Patients with Neurofibromatosis Type 1

Abstract

In various studies short telomere length and low telomerase activity have been shown to have a protective role in some types of cancer. Patients with NF1 are under risk of developing certain types of cancer. Malignant tumors such as brain tumors, malignant peripheral nerve sheat tumors, optic gliomas, soft tissue sarcomas and benign tumors such as neurofibromas and hamartomas are among them. Interestingly, prognosis is generally better in patients with NF1 than those without NF1. In the present review, we aimed to delineate telomerase activity in patients with NF1 with various types of cancer. Measurement of telomerase activity in patients with NF1 can be a diagnostic marker for tumorigenesis.

Keywords

Neurofibromatosis Type 1, telomerase activity, tumors

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