Comparison of Polystemia Vera Patients Diagnosed Before and After the Updated Diagnostic Criteria

Yıl 2024, Cilt: 46 Sayı: 3, 333 - 343, 27.05.2024

Şevket Katılmış , Eren Gunduz

https://doi.org/10.20515/otd.1413126

Öz

Polycythemia vera is a chronic, clonal and progressive myeloproliferative disease characterized by leukocytosis, thrombocytosis and splenomegaly with increased erythroid series. In this study, we aimed to compare the clinical and laboratory features of patients diagnosed with PV according to the 2008 and 2016 WHO diagnostic criteria and the complications that developed during follow-up, and to retrospectively evaluate the impact of the change in diagnostic criteria on real life. A total of 100 polycythemia vera patients with positive JAK2-V617F mutation were enrolled in the study, 50 between 2011-2015 and 50 between 2016-2020. When the two groups were compared in terms of laboratory values at the time of diagnosis, hemoglobin, hematocrit and red blood cell count were significantly higher in the group diagnosed before 2016 (p=0.036). When patients were evaluated for complications of thrombosis, hemorrhage, progression to myelofibrosis, leukemic transformation, and transformation of myelodysplastic syndrome at last follow-up, it was found that 19 (38%) patients in the pre-2016 group and 5 (10%) patients in the post-2016 group developed complications (p=0.002). The number of patients who developed a myelofibrosis complication was significantly higher in the pre-2016 group (p=0.006). The number of patients who developed thrombosis was higher in the pre-2016 group, but the difference did not reach a statistically significant level (p=0.055). About other complications, there was no difference between the two groups (p > 0.05). In summary, our study found a significant difference only in the number of patients with myelofibrosis complications. Further studies with more patients will be needed to determine the impact of the 2016 change in diagnostic criteria on other complications.

Anahtar Kelimeler

Polystemia vera, Diagnosis, Treatment, Complications

Etik Beyan

This study is an original study; preparation, data collection, analysis and presentation of information in accordance with the principles and rules of scientific ethics for all data and information not obtained within the scope of this study. and that I have included these sources in the bibliography; that I have not made any errors in the data used I have not made any changes, the study meets all the requirements of the Committee on Publication Ethics (COPE) I declare that I comply with ethical duties and responsibilities by accepting the terms and conditions of the study. At any time, I declare that any situation contrary to this statement I made about the study I consent to all moral and legal consequences in the event of detection I'll let you know.

Teşekkür

We would like to thank Professor Ertuğrul ÇOLAK from the Department of Biostatistics for his support during the data analysis and statistical phase of our study.

Güncellenen Tanı Kriterleri Öncesi ve Sonrası Tanı Alan Polistemia Vera Hastalarının Karşılaştırılması

Öz

Polisitemia vera, eritroid serilerde artışla birlikte lökositoz, trombositoz ve splenomegali ile karakterize kronik, klonal ve ilerleyici bir miyeloproliferatif hastalıktır. Bu çalışmada, 2008 ve 2016 DSÖ tanı kriterlerine göre PV tanısı alan hastaların klinik ve laboratuvar özellikleri ile takip sırasında gelişen komplikasyonları karşılaştırmayı ve tanı kriterlerindeki değişikliğin gerçek hayata etkisini retrospektif olarak değerlendirmeyi amaçladık. Çalışmaya 2011-2015 yılları arasında 50 ve 2016-2020 yılları arasında 50 olmak üzere JAK2-V617F mutasyonu pozitif olan toplam 100 polisitemia vera hastası dahil edilmiştir. İki grup tanı anındaki laboratuvar değerleri açısından karşılaştırıldığında, hemoglobin, hematokrit ve kırmızı kan hücresi sayısı 2016'dan önce tanı alan grupta anlamlı olarak daha yüksekti (p=0,036). Hastalar son takipte tromboz, kanama, miyelofibrozise ilerleme, lösemik dönüşüm ve miyelodisplastik sendroma dönüşüm komplikasyonları açısından değerlendirildiğinde, 2016 öncesi grupta 19 (%38) hastada, 2016 sonrası grupta ise 5 (%10) hastada komplikasyon geliştiği tespit edilmiştir (p=0,002). Miyelofibrozis komplikasyonu gelişen hasta sayısı 2016 öncesi grupta anlamlı olarak daha yüksekti (p=0,006). Tromboz gelişen hasta sayısı 2016 öncesi grupta daha yüksekti, ancak fark istatistiksel olarak anlamlı düzeye ulaşmadı (p=0,055). Diğer komplikasyonlar açısından iki grup arasında fark bulunmamıştır (p > 0,05). Özet olarak, çalışmamızda sadece miyelofibrozis komplikasyonu görülen hasta sayısında anlamlı bir fark bulunmuştur. Tanı kriterlerinde 2016'da yapılan değişikliğin diğer komplikasyonlar üzerindeki etkisini belirlemek için daha fazla hasta ile daha ileri çalışmalara ihtiyaç duyulacaktır.

Anahtar Kelimeler

Polistemia vera, Tanı, Tedavi, Komplikasyon

Etik Beyan

Bu çalışmanın, özgün bir çalışma olduğunu; çalışmanın hazırlık, veri toplama, analiz ve bilgilerin sunumu olmak üzere tüm aşamalarından bilimsel etik ilke ve kurallarına uygun davrandığımı; bu çalışma kapsamında elde edilmeyen tüm veri ve bilgiler için kaynak gösterdiğimi ve bu kaynaklara kaynakçada yer verdiğimi; kullanılan verilerde herhangi bir değişiklik yapmadığımı, çalışmanın Committee on Publication Ethics (COPE)' in tüm şartlarını ve koşullarını kabul ederek etik görev ve sorumluluklara riayet ettiğimi beyan ederim. Herhangi bir zamanda, çalışmayla ilgili yaptığım bu beyana aykırı bir durumun saptanması durumunda, ortaya çıkacak tüm ahlaki ve hukuki sonuçlara razı olduğumu bildiririm.

Teşekkür

Çalışmamızın verilerin analizi ve istatistik aşamasında destek veren biyoistatistik anabilim dalından Profesör doktor Ertuğrul ÇOLAK' a teşekkür ederiz.

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