Esansiyel trombositozlu hastaların kemik iliği biyopsisi ve myelofibrosize transformasyonun retrospektif incelenmesi
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Dosyalar
Tarih
2024
Kurum Yazarları
Akduman, Resul
Süreli Yayın başlığı
Süreli Yayın ISSN
Cilt Başlığı
Yayınevi
Bursa Uludağ Üniversitesi
Özet
Esansiyel trombositoz (ET); periferik kanda trombosit sayısının artışı ile seyreden kronik myeloproliferatif hastalıktır (KMPH). ET tanılı 201 hastadan oluşan rektrospektif çalışmamızda; hastalığın komplikasyonlarını, kemik iliği biyopsi sonuçlarını, myelofibrozise(MF) dönüşümünü değerlendirmeyi amaçladık. Çalışmaya, Bursa Uludağ Üniversitesi Tıp Fakültesi İç Hastalıkları Ana Bilim Dalı, Hematoloji Bilim Dalı 2012 Aralık ile 2022 Aralık tarihleri arasında başvurmuş ET tanısı olan 201 erişkin hasta dahil edildi. Hastaların demografik özellikleri, kemik iliği biyopsi fibrozis dereceleri, sitogenetik JAK2 sonuçları, The Endothelial Activation and Stress Index (EASIX) skoru retrospektif olarak tarandı. ET tanılı hastaların medyan tanı yaşı 51 yıldı. Hastaların %66,7' si (n=134) kadındı. Hastaların %7,50'sinde (n=15) tanı anında tromboz mevcuttu. Bir hastada tedavi altında tromboz, iki hastada kanama gelişti. MF dönüşüm oranı %6,10 (n=12) olarak saptandı. MF dönüşüm yılı için medyan süre değeri 7,5 yıl olarak hesaplandı. MF dönüşümü gözlenen 12 hastada 2 tanesine allojeneik kemik iliği nakli yapıldı. Akut myeloid lösemiye dönüşüm gözlenen 2 hasta saptandı. MF'ye dönüşen ve dönüşmeyen hasta grupları karşılaştırıldığında laktat dehidrogenaz (LDH) değeri dönüşen grupta yüksek bulundu (p<0,001). Bundan dolayı ET ve post ET MF tanılı hastalarda EASIX ve türevleri hesaplandı, MF'ye dönüşen hastalarda anlamlı yükseklik görüldü. Yine MF'ye dönüşüm için risk analizi sonucunda hemoglobin <12,5 g/DL olan hasta grubunda MF dönüşüm riski 11,33 kat (p<0,028), tedaviye yanıt kaybı gelişen grupta 39.06 kat (p<0,001) ve modifiye simple EASIX skoru >0,43 üstünde olan grupta MF dönüşüm riski 30,77 kat (p<0,001) daha fazla olduğu belirlendi. Sonuç olarak çalışmamız ET'nin myelofibrozise dönüşümü için, LDH yüksekliği ve EASIX skorunun ön gördürücü olabileceğini düşündürmektedir.
Essential thrombocytosis (ET) is a chronic myeloproliferative disease (CMPD) characterised by increased platelet count in peripheral blood. In our retrospective study of 201 patients diagnosed with ET, we aimed to evaluate the complications of the disease, bone marrow biopsy results and transformation to myelofibrosis (MF). The study was carried out with 201 patients with ET who were admitted to Bursa Uludag University Faculty of Medicine, Department of Internal Medicine, Division of Hematology between December 2012 and December 2022. The patients' files, epicrises and hospital data processing system were reviewed retrospectively. The median age at diagnosis of ET was 51 years. 66.7% (n=134) of the patients were female. Thrombosis was present in 7.50% (n=15) of the patients at the time of diagnosis. One patient developed thrombosis and two patients developed haemorrhage under treatment. MF transformation rate was 6.10% (n=12). The median time to MF transformation was calculated as 7.5 years. Allogeneic bone marrow transplantation was performed in 2 of 12 patients with MF transformation. Transformation to acute myeloid leukaemia was observed in 2 patients. When the groups of patients with and without transformation to MF were compared, the lactate dehydrogenase (LDH) value was found to be higher in the transformation group (p<0.001). Therefore, we calculated The Endothelial Activation and Stress Index (EASIX) and its derivatives in patients with ET and post-ET MF, and a significant increase was observed in patients who converted to MF. As a result of multivariate logistic regression analysis in which we examined the risk factors for transformation to MF, the risk of transformation to MF was 11.33 times (p<0.028) higher in the group of patients with haemogram <12.5g/DL, 39.06 times (p<0.001) higher in the group with loss of response to treatment, and 30.77 times (p<0.001) higher in the group with modified simple EASIX score >0.43. In conclusion, we think that LDH elevation and EASIX score may be predictive of this transformation to myelofibrosis.
Essential thrombocytosis (ET) is a chronic myeloproliferative disease (CMPD) characterised by increased platelet count in peripheral blood. In our retrospective study of 201 patients diagnosed with ET, we aimed to evaluate the complications of the disease, bone marrow biopsy results and transformation to myelofibrosis (MF). The study was carried out with 201 patients with ET who were admitted to Bursa Uludag University Faculty of Medicine, Department of Internal Medicine, Division of Hematology between December 2012 and December 2022. The patients' files, epicrises and hospital data processing system were reviewed retrospectively. The median age at diagnosis of ET was 51 years. 66.7% (n=134) of the patients were female. Thrombosis was present in 7.50% (n=15) of the patients at the time of diagnosis. One patient developed thrombosis and two patients developed haemorrhage under treatment. MF transformation rate was 6.10% (n=12). The median time to MF transformation was calculated as 7.5 years. Allogeneic bone marrow transplantation was performed in 2 of 12 patients with MF transformation. Transformation to acute myeloid leukaemia was observed in 2 patients. When the groups of patients with and without transformation to MF were compared, the lactate dehydrogenase (LDH) value was found to be higher in the transformation group (p<0.001). Therefore, we calculated The Endothelial Activation and Stress Index (EASIX) and its derivatives in patients with ET and post-ET MF, and a significant increase was observed in patients who converted to MF. As a result of multivariate logistic regression analysis in which we examined the risk factors for transformation to MF, the risk of transformation to MF was 11.33 times (p<0.028) higher in the group of patients with haemogram <12.5g/DL, 39.06 times (p<0.001) higher in the group with loss of response to treatment, and 30.77 times (p<0.001) higher in the group with modified simple EASIX score >0.43. In conclusion, we think that LDH elevation and EASIX score may be predictive of this transformation to myelofibrosis.
Açıklama
Anahtar kelimeler
Esansiyel trombositoz, Myelofibrozis, LDH yüksekliği, EASIX skoru, Essential thrombocytosis, Myelofibrosis, LDH elevation, EASIX score
Alıntı
Akduman, R. (2024). Esansiyel trombositozlu hastaların kemik iliği biyopsisi ve myelofibrosize transformasyonun retrospektif incelenmesi. Yayınlanmamış tıpta uzmanlık tezi. Bursa Uludağ Üniversitesi Tıp Fakültesi.