WHO 2016 Prefibrotic Myelofibrosis in the Patients with WHO 2008 Essential Thrombocythemia

Aktimur S. H., Baycelebi D., Gunes A. K., Yıldız L., Turgut M.

UHOD-ULUSLARARASI HEMATOLOJI-ONKOLOJI DERGISI, cilt.31, sa.3, ss.139-145, 2021 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 31 Sayı: 3
  • Basım Tarihi: 2021
  • Doi Numarası: 10.4999/uhod.215031
  • Dergi Adı: UHOD-ULUSLARARASI HEMATOLOJI-ONKOLOJI DERGISI
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, EMBASE, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.139-145
  • Anahtar Kelimeler: Essential thrombocythemia, Primary myelofibrosis, Anemia, Splenomegaly, Progression, Bone marrow, WORLD-HEALTH-ORGANIZATION, MYELOID NEOPLASMS, CLASSIFICATION, MUTATIONS, DIAGNOSIS, REVISION, RECEPTOR
  • Ondokuz Mayıs Üniversitesi Adresli: Evet

Özet

According to newly defined features of bone marrow (BM) histology, some of the patients who were previously diagnosed with essential thrombocythemia (ET) were accepted as early/prefibrotic PMF in recent publications. The aim of this study was to explore actual rate of pre-PMF according to the 2016 revised WHO criteria. Demographic characteristics of 160 patients diagnosed with ET between 2000-2017; laboratory values; cytogenetic profile; the treatments using; disease-related thromboembolic complications; progression of the disease to MF and AML; mortality rates and cause were recorded retrospectively. The diagnosis of pre-PMF or ET was confirmed by BM morphology and clinical follow-up. BM biopsy samples obtained during the initial diagnosis were available in 107 cases. 53 cases with inaccessible BM biopsies were excluded from the study. The distribution of female/male in cases of ET was 46/28. The incidence of progression to AML was higher in pre-PMF patients. (Progression to AML: PMF 15.15%; ET 4.05%; p= 0.044). The mean duration of progression-free survival in patients with progression to AML or PMF in pre-PMF patients was 52.1 +/- 7.37 months; ET was 62.44 +/- 10.20 months. Approximately 30% of patients previously diagnosed with ET consisted of pre-PMF patients. Anemia, high LDH level, and splenomegaly are parameters that can be used in the differential diagnosis of PMF.