SavedAdd your journal

Journals / Osmangazi Tıp Dergisi / 2026 / Cilt: 46 Sayı: 3

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

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

Şevket Katılmış(ESKİŞEHİR OSMANGAZI UNIVERISTY FACULTY OF MEDICINE, DEPARTMENT OF INTERNAL MEDICINE)0009-0005-1132-7716
Eren Gunduz(ESKİŞEHİR OSMANGAZI UNIVERSTY FACULTY OF MEDICINE, DEPARTMENT OF INTERNAL MEDICINE, DEPARTMENT OF HAEMATOLOGY)0000-0001-7455-2949
Pages
333–343
Publication Date
2024-05-27
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

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).

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Polycythemia vera is a chronic, clonal and progressive myeloproliferative disease characterized by leukocytosis, thrombocytosis and splenomegaly with increased erythroid series.
📊
Key Findings & Data: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.
💡
Conclusion & Impact: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).
🔬Design:Araştırma Makalesi
📍Scope:2008 Verileri
⚙️Variables:Polistemia vera · Tanı · Tedavi

Abstract

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.

Özet

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.

🔖

Cite This Article

Katılmış, Ş., Gunduz, E. (2024). Comparison of Polystemia Vera Patients Diagnosed Before and After the Updated Diagnostic Criteria. Osmangazi Tıp Dergisi, Cilt: 46 Sayı: 3, 333–343. https://doi.org/10.20515/otd.1413126

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (44)

  1. Spivak JL, Barosi G, Tognoni G, Barbui T, Finazzi G, Marchioli R, et al. Chronic myeloproliferative disorders. Hematology American Society of Hematology Education Program. 2003:200-24.
  2. Gianelli U, Iurlo A, Vener C, Moro A, Fermo E, Bianchi P, et al. The significance of bone marrow biopsy and JAK2 V617F mutation in the differential diagnosis between the “early” prepolycythemic phase of polycythemia vera and essential thrombocythemia. 2008;130(3):336-42.
  3. Johansson PL, Safai‐Kutti S, Kutti JJBjoh. An elevated venous haemoglobin concentration cannot be used as a surrogate marker for absolute erythrocytosis: a study of patients with polycythaemia vera and apparent polycythaemia. 2005;129(5):701-5.
  4. Alvarez-Larrán A, Ancochea A, Angona A, Pedro C, García-Pallarols F, Martínez-Avilés L, et al. Red cell mass measurement in patients with clinically suspected diagnosis of polycythemia vera or essential thrombocythemia. 2012;97(11):1704.
  5. Barbui T, Thiele J, Carobbio A, Guglielmelli P, Rambaldi A, Vannucchi AM, et al. Discriminating between essential thrombocythemia and masked polycythemia vera in JAK2 mutated patients. 2014;89(6):588-90.
  6. Silver RT, Chow W, Orazi A, Arles SP, Goldsmith SJJB, The Journal of the American Society of Hematology. Evaluation of WHO criteria for diagnosis of polycythemia vera: a prospective analysis. 2013;122(11):1881-6.
  7. Maffioli M, Mora B, Passamonti FJCAHO. Polycythemia vera: from new, modified diagnostic criteria to new therapeutic approaches. 2017;15(9):700-7.
  8. Barbui T, Thiele J, Passamonti F, Rumi E, Boveri E, Randi ML, et al. Initial bone marrow reticulin fibrosis in polycythemia vera exerts an impact on clinical outcome. 2012;119(10):2239-41.
  9. Passamonti F, Pietra D, Rumi E, Arcaini L, Della Porta M, Malcovati L, et al. PRV-1 and its correlation with treatments and disease status in 210 patients with polycythemia vera and essential thrombocythemia. 2005;19(5):888-9.
  10. Passamonti F, Vanelli L, Malabarba L, Rumi E, Pungolino E, Malcovati L, et al. Clinical utility of the absolute number of circulating CD34-positive cells in patients with chronic myeloproliferative disorders. 2003;88(10):1123-9.
  11. Passamonti F, Maffioli M, Caramazza D, Cazzola MJO. Myeloproliferative neoplasms: from JAK2 mutations discovery to JAK2 inhibitor therapies. 2011;2(6):485.
  12. Pietra D, Li S, Brisci A, Passamonti F, Rumi E, Theocharides A, et al. Somatic mutations of JAK2 exon 12 in patients with JAK2 (V617F)-negative myeloproliferative disorders. 2008;111(3):1686-9.
  13. Silver RT, Krichevsky S, Gjoni S, Cross NCJL, lymphoma. Evaluation of serum erythropoietin values as defined by 2016 World Health Organization criteria for the diagnosis of polycythemia vera. 2017;58(11):2768-9.
  14. Barbui T, Finazzi G, Falanga AJB, The Journal of the American Society of Hematology. Myeloproliferative neoplasms and thrombosis. 2013;122(13):2176-84.
  15. Noor SJ, Tan W, Wilding GE, Ford LA, Barcos M, Sait SN, et al. Myeloid blastic transformation of myeloproliferative neoplasms—A review of 112 cases. 2011;35(5):608-13.
  16. Kiladjian JJ, Rain JD, Bernard JF, Briere J, Chomienne C, Fenaux P. Long-term incidence of hematological evolution in three French prospective studies of hydroxyurea and pipobroman in polycythemia vera and essential thrombocythemia. Semin Thromb Hemost. 2006 Jun;32(4 Pt 2):417-21.
  17. Tefferi A, Guglielmelli P, Larson DR, Finke C, Wassie EA, Pieri L, et al. Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis. 2014;124(16):2507-13.
  18. Tefferi A, Rumi E, Finazzi G, Gisslinger H, Vannucchi A, Rodeghiero F, et al. Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study. 2013;27(9):1874-81.
  19. Iurlo A, Cattaneo D, Bucelli C, Baldini LJIJoMS. New perspectives on polycythemia vera: from diagnosis to therapy. 2020;21(16):5805.
  20. Sultan S, Irfan SM, Murad S. Clinico-Epidemiological Profile of Patients with Polycythaemia Rubra Vera - a Five Year Experience from a Tertiary Care Center. Asian Pacific journal of cancer prevention: APJCP. 2016;17(3):1531-3.
  21. Özkan A. Erı̇şkı̇n hematolojı̇ polı̇klı̇nı̇ğı̇ne başvuran polı̇sı̇temı̇ vera tanılı hastaların klı̇nı̇k ve laboratuvar parametrelerı̇nı̇n değerlendı̇rı̇lmesı̇ [tıpta uzmanlık tezi]: Dicle Üniversitesi; 2019.
  22. Parasuraman S, Yu J, Paranagama D, Shrestha S, Wang L, Baser O, et al. Cytoreductive treatment patterns among US veterans with polycythemia vera. 2018;18(1):1-7.
  23. Finazzi GJPB. A prospective analysis of thrombotic events in the European collaboration study on low-dose aspirin in polycythemia (ECLAP). 2004;52(5):285-8.
  24. S. KY. Polisitemia Vera ve İzole Hemoglobin/Hematokrit Yüksekliği [İç Hastalıkları Uzmanlık Tezi]: Hacettepe Üniverstitesi; 2019.
  25. Anger B, Haug U, Seidler R, Heimpel HJB. Polycythemia vera. A clinical study of 141 patients. 1989;59(6):493-500.
  26. Landolfi R, Marchioli R. European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP): a randomized trial. Seminars in thrombosis and hemostasis. 1997;23(5):473-8.
  27. Eyüpler Mercan Ç. Polisitemia vera hastalarinin demografik ve klinik özelliklerinin survi ve prognoz üzerine etkisinin incelenmesi: retrospektif tek merkez deneyimi [Tıpta uzmanlık tezi ]: Marmara üniversitesi; 2021.
  28. Elliott MA, Tefferi AJBjoh. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. 2005;128(3):275-90.
  29. Shaikh MS, Shaikh MU, Adil SN, Khurshid M, Ahmed ZA. Clinico-Pathological Profile And Outcomes Of Patients With Polycythaemia Vera, Essential Thrombocythaemia And Idiopathic Myelofibrosis: A Tertiary Care Center Experience From Southern Pakistan. Journal of Ayub Medical College, Abbottabad: JAMC. 2016;28(2):293-7.
  30. Landolfi R, Marchioli R, Kutti J, Gisslinger H, Tognoni G, Patrono C, et al. Efficacy and safety of low-dose aspirin in polycythemia vera. 2004;350(2):114-24.
  31. Marchioli R, Finazzi G, Landolfi R, Kutti J, Gisslinger H, Patrono C, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. Journal of clinical oncology: official journal of the American Society of Clinical Oncology. 2005;23(10):2224-32.
  32. Passamonti F, Rumi E, Pungolino E, Malabarba L, Bertazzoni P, Valentini M, et al. Life expectancy and prognostic factors for survival in patients with polycythemia vera and essential thrombocythemia. 2004;117(10):755-61.
  33. Policitemia GISJAoIM. Polycythemia vera: the natural history of 1213 patients followed for 20 years. 1995;123(9):656-64.
  34. Landolfi R, Marchioli R, editors. European collaboration on low-dose aspirin in polycythemia vera (ECLAP): a randomized trial. Seminars in thrombosis and hemostasis; 1997: Copyright© 1997 by Thieme Medical Publishers, Inc.
  35. Marchioli R, Finazzi G, Landolfi R, Kutti J, Gisslinger H, Patrono C, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. 2005;23(10):2224-32.
  36. Schafer AI. Bleeding and thrombosis in the myeloproliferative disorders. 1984.
  37. Landolfi R, Rocca B, Patrono CJCrioh. Bleeding and thrombosis in myeloproliferative disorders: mechanisms and treatment. 1995;20(3):203-22.
  38. Bai J, Ai L, Zhang L, Yang FC, Zhou Y, Xue YJAjoh. Incidence and risk factors for myelofibrotic transformation among 272 Chinese patients with JAK2‐mutated polycythemia vera. 2015;90(12):1116-21.
  39. Gangat N, Strand J, Li CY, Wu W, Pardanani A, Tefferi AJBjoh. Leucocytosis in polycythaemia vera predicts both inferior survival and leukaemic transformation. 2007;138(3):354-8.
  40. Crisà E, Venturino E, Passera R, Prina M, Schinco P, Borchiellini A, et al. A retrospective study on 226 polycythemia vera patients: impact of median hematocrit value on clinical outcomes and survival improvement with anti-thrombotic prophylaxis and non-alkylating drugs. 2010;89(7):691-9.
  41. Passamonti F, Rumi E, Caramella M, Elena C, Arcaini L, Boveri E, et al. A dynamic
  42. Sultan S, Irfan SM, Murad S. Clinico-Epidemiological Profile of Patients with Polycythaemia Rubra Vera - a Five Year Experience from a Tertiary Care Center. Asian Pacific journal of cancer prevention: APJCP. 2016;17(3):1531-3.
  43. Shaikh MS, Shaikh MU, Adil SN, Khurshid M, Ahmed ZA. Clinico-Pathological Profile And Outcomes Of Patients With Polycythaemia Vera, Essential Thrombocythaemia And Idiopathic Myelofibrosis: A Tertiary Care Center Experience From Southern Pakistan. Journal of Ayub Medical College, Abbottabad: JAMC. 2016;28(2):293-7.
  44. Marchioli R, Finazzi G, Landolfi R, Kutti J, Gisslinger H, Patrono C, et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. Journal of clinical oncology: official journal of the American Society of Clinical Oncology. 2005;23(10):2224-32.