Dergiler / Uluslararası Hematoloji-Onkoloji Dergisi / 2019 / Cilt: 29 - Sayı: 1

Fibrosis Development, Leukemic Transformation and Secondary Malignancies Complicating the Clinical Course of Essential Thrombocythemia

Esansiyel Trombositozun Klinik Seyrini Oluşturan Fibrozis Gelişimi, Lösemik Dönüşüm ve Sekonder Malignitelerin Değerlendirilmesi

Sayfa
14–21
DOI
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Moreover, ET patients who had received HU and anagrelide treatment has better OS than the other patient cohorts.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Essential thrombocythemia (ET) is a myeloproliferative neoplastic disease characterized by abnormal proliferation of megakaryocytes in the bone marrow leading to elevated platelet counts in the peripheral blood.
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Temel Bulgular & Veri:There were 75 (60.5%) men and 49 (39.5%) women with a median age of 53 (range, 20–80) years.
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Sonuç & Katkı:Moreover, ET patients who had received HU and anagrelide treatment has better OS than the other patient cohorts.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Özet

Essential thrombocythemia (ET) is a myeloproliferative neoplastic disease characterized by abnormal proliferation of megakaryocytes in the bone marrow leading to elevated platelet counts in the peripheral blood. In the present study, we aim to assess the long-term complications of ET and its treatments in terms of the development of secondary malignancies, bone marrow fibrosis and leukemic transformation. One hundred and twenty four patients with ET were included into the study. Retrospective data were collected from our database of myeloproliferative disorders. There were 75 (60.5%) men and 49 (39.5%) women with a median age of 53 (range, 20–80) years. The data indicated that 3 patients treated with hydroxyurea (HU) had suffered of bladder cancer, non-small cell lung cancer and thyroid papillary cancer, 2 patients without treatment suffered of breast cancer and neuroendocrine cancer and 2 patients treated with combination (HU and anagrelide) suffered of acute myeloid leukemia (AML) and prostate cancer. In total, 16/124 patients (12.9%) developed bone marrow fibrosis. Nine of the patients (7.2%) who developed bone marrow fibrosis were receiving HU, 5 of them (4%) were using HU and anagrelide and 2 of them (1.6%) were followed without treatment (p= 0.44). The age (p= 0.03), hemoglobin level at diagnosis (p< 0.001), white blood cell level at diagnosis (p= 0.02), CRP level (p= 0.01), pre-treatment hemorrhage rate (p< 0.001) were statistically significant different between the patients who developed myelofibrosis and patients who did not develop myelofibrosis. The results of this study disclosed that there was no statistically significant difference regarding the development of bone marrow fibrosis, leukemic transformation or secondary malignancies with regard to the treatment options of ET. Moreover, ET patients who had received HU and anagrelide treatment has better OS than the other patient cohorts.

Abstract

Esansiyel trombositemi (ET), periferik kanda trombosit sayısında artışa yol açan kemik iliğinde anormal megakaryosit proliferasyonu ile karakterize miyeloproliferatif bir neoplastik hastalıktır. Bu çalışmada ET’nin uzun süreli komplikasyonlarını ve tedavilerini sekonder maligniteler, kemik iliği fibrozu ve lösemi transformasyonu açısından değerlendirmeyi amaçladık. ET’li 124 hasta çalışmaya dahil edildi. Retrospektif veriler, myeloproliferatif hastalıklar veritabanımızdan edinilmiştir. Hastaların ortanca yaşı 53 (20-80) saptandı. Çalışmaya 75 (%60.5) erkek ve 49 (% 39,5) kadın dahil edildi. Hidroksiüre (HU) ile tedavi edilen 3 hastada mesane kanseri, küçük hücreli dışı akciğer kanseri ve tiroid papiller kanseri gözlendi. Tedavi almayan 2 hastada meme kanseri ve nöroendokrin kanseri gelişti. Hidroksiüre ve anagrelide alan 2 hastada AML ve prostat kanseri izlendi. Toplamda 16 hastada (%12.9) kemik iliği fibrozu gelişti. Kemik iliği fibrozisi gelişen hastaların 9’u (%7.2) HU, 5’i (% 4) HU ve anagrelid kullanıyordu, 2 (%1.6) hasta ise ilaçsız takip ediliyordu (p= 0.44). Yaş (p= 0.03), tanıdaki hemoglobin düzeyi (p< 0.001), beyaz küre sayısı (p= 0.02), CRP düzeyi (p= 0.01) ve tedavi başlanmadan önceki kanama oranı (p< 0.001) takipte myelofibrosis gelişen hastalarla, myelofibrosis gelişmeyen hastalar arasında istatistiksel olarak anlamlı farklılık gözlendi. Bu çalışmanın sonuçları, ET’de tedavi seçenekleri açısından kemik iliği fibrozu, lösemik transformasyon veya sekonder malignite gelişimi açısından istatistiksel olarak anlamlı bir fark olmadığını ortaya koydu. Ayrıca, HU ve anagrelid tedavisi alan ET hastaları diğer hasta gruplarından daha iyi OS’ye sahipti.

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Bu Makaleye Atıf Yap

ÇİFTÇİLER, R., AKSU, S., MALKAN, Ü. Y., BÜYÜKAŞIK, Y., HAZNEDAROĞLU, İ. C. (2019). Fibrosis Development, Leukemic Transformation and Secondary Malignancies Complicating the Clinical Course of Essential Thrombocythemia. Uluslararası Hematoloji-Onkoloji Dergisi, Cilt: 29 - Sayı: 1, 14–21. https://ajindex.com/articles/322749

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