Ruxolitinib use in myelofibrosis patients: the single center experience and the relationship between JAK-2 allele burden and Ruxolitinib response

KURTOĞLU ,Erdal; GÖÇER ,Mesut

doi:10.31362/patd.770631

Erdal KURTOĞLU

(Antalya Eğitim ve Araştırma Hastanesi, İç Hastalıkları Anabilim Dalı, Hematoloji Bilim Dalı, Antalya, Türkiye)

Mesut GÖÇER

(Antalya Eğitim ve Araştırma Hastanesi, İç Hastalıkları Anabilim Dalı, Hematoloji Bilim Dalı, Antalya, Türkiye)

Pamukkale Tıp Dergisi

Yıl: 2021Cilt: 14Sayı: 2ISSN: 1309-9833 / 1308-0865Sayfa Aralığı: 322 - 329Metin Dili: İngilizce

DOI: https://doi.org/10.31362/patd.770631

48 0

İngilizce
Türkçe

Ruxolitinib use in myelofibrosis patients: the single center experience and the relationship between JAK-2 allele burden and Ruxolitinib response

Abstract: Purpose: Ruxolitinib is an oral JAK-1/2 inhibitor approved for the treatment of splenomegaly and/or constitutionalsymptoms in intermediate and high-risk myelofibrosis patients. The aim of our study is to evaluate the efficacyand safety of ruxolitinib in primary MF, post-ET MF and post-PV MF patients, to evaluate the relationshipbetween response and JAK-2 allele burden and to compare them with literature data.Materials and methods: In our single centered and retrospective study, we investigated the data of 30 MFpatients diagnosed in our clinic between May 2015 and December 2019. We reported demographic features,laboratory values, and spleen sizes.Results: 18 patients (60%) with a median age of 67.5 (45-78) had primary myelofibrosis. Spleen sizes decreasedsignificantly 3 and 6 months after treatment. Constitutional symptoms have disappeared in 28 patients (93.3%).No association was found between JAK-2 allele burden and treatment response success.Conclusion: Ruxolitinib MF is very safe and effective to relieve constitutional symptoms and decrease spleensize. Despite JAK-2 inhibition, no linear relationship was found between JAK-2 allele burden and treatmentefficacy.

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Subject Area:

Myelofibrozis hastalarında Ruxolitinib kullanımı: tek merkez deneyimi ve JAK-2 allel yükü ile Ruxolitinib yanıtı arasındaki ilişki

Öz:Amaç: Ruksolitinib, intermediate ve yüksek risk myelofibrozis hastalarında splenomegali ve/veya konstitusyonel semptomların tedavisi için onay almış oral olarak kullanılan bir JAK-1/2 inhibitörüdür. Bu çalışmamızda amacımız primer MF, post-ET MF ve post-PV MF hastalarında ruksolitinibin etkinlik ve güvenirliliğini değerlendirmek, JAK2 allel yüküyle yanıt ilişkisini değerlendirmek ve literatür verileriyle karşılaştırmaktır. Gereç ve yöntem: Tek merkezli ve retrospektif çalışmamızda kliniğimizde Mayıs 2015 ile Aralık 2019 tarihleri arasında tanı almış 30 MF hastasının verilerini dosyalarından inceledik. Demografik özelliklerini, laboratuvar değerlerini ve dalak boyutlarını kaydettik. Bulgular: Mediyan yaşları 67,5 (45-78) olan hastaların 18 tanesi (%60) primer myelofibrozis hastasıydı. Ruksolitinib tedavisi sonrası 3.ay ve 6.ayda hastaların dalak boyutlarında anlamlı azalma saptandı. Hastaların 28 tanesinin (%93,3) konstitusyonel semptomları kayboldu. JAK-2 allel yüküyle tedavi yanıt başarısı arasında ilişki saptanmadı. Tartışma: Ruxolitinib MF tedavisinde hem konstitusyonel semptomları ortadan kaldırmada hem de dalak boyutunda azalma sağlamada oldukça etkin ve güvenlidir. JAK-2 inhibisyonu yapmasına rağmen JAK-2 allel yükü ile tedavi etkinliği arasında ise lineer bir ilişki yoktur.

Anahtar Kelimeler:

Konu Alanı:

Belge Türü: DergiMakale Türü: Araştırma MakalesiErişim Formatı: Erişime Açık

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