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Vol. 42. Issue S1.
Pages 19 (October 2020)
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Vol. 42. Issue S1.
Pages 19 (October 2020)
OP 04
Open Access
Analysis of demographic and clinical characteristics of primary myelofibrosis and post-polycythemia vera/essential thrombocythemia myelofibrosis patients
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P. Akyol, A. Yıldız, M. Albayrak, H. Afacan Öztürk, S. Maral, M. Reis Aras*, F. YıLmaz, B. Saglam, M. Tıglıoglu, U. Malkan
Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey
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Objective: Myelofibrosis (MF), could be de novo (primary myelofibrosis [PMF]), as well as developing in the clinical course of polycythemia vera (PV) or essential thrombocythemia (ET). PMF and post-PV/ET MF have many common features with clinical course and laboratory findings. However, there are insufficient studies showing the etiological or morphological differences between these patients. In this context, the aim of this study was to contribute to the literature by comparing PMF and PV/ET patients who developed MF.

Case report:

Methodology: This retrospective study included 31 patients who were diagnosed with PMF and post-PV/ET MF in the Hematology Department of Dışkapı Yıldırım Beyazıt Training and Research Hospital between 2008–2019. The diagnosis of PMF was made according to the WHO criteria and the IWG-MRT group criteria were used for the diagnosis of PPV-MF and PET-MF. The two groups were compared in terms of demographic and clinical features. The diagnosis date, demographic and clinical features, physical examination findings, mutation analyses, treatment management and follow-up times of all the patients were recorded. Hematological parameters including Hb, hematocrit (Hct), leukocyte (WBC), neutrophil, lymphocyte, monocyte, platelet, platelet distribution width (PDW), mean platelet volume (MPV), LDH, ferritin and Vitamin B12 levels were examined.

Results: Evaluation was made of a total of 31 patients, including 16 PMF and 15 post-PV/ET MF. The mean follow-up period was 31.1 months [1–107.5]. JAK-2 V617F gene mutation was detected 10 (62.5%) PMF patients and 12 (80%) post-PV/ET MF patients. Splenomegaly was detected at the time of diagnosis in all PMF and post-PV/ET MF patients. When the size of the spleen was examined, there was no statistically significant difference between the two groups.JAK-2 V617F gene mutation was detected 10 (62.5%) PMF patients and 12 (80%) post-PV/ET MF patients. In terms of JAK-2 V617F mutation positivity, there was no statistically significant difference between the two groups. JAK-2 V617F mutation, and allele burden of ≥60% was detected in 70% of PMF patients and in 90% of post-PV/ET MF patients. The allele burden was not determined to affect OS in patients with MF. Hydroxyurea was most frequently used as the first line treatment in PMF (81.3%), while ruxolitinib was preferred in post ET/PV MF (53.3%). Throughout the follow-up period, thromboembolic complications developed in 12.5% of PMF patients and in 13.3% of post-PV/ET MF patients. There was no statistically significant difference between the two groups in terms of thromboembolic complications. Acute myeloid leukemia transformation was observed in 1 (6.25%) patient from the PMF group during the follow-up period. The OS of patients was mean 63.6 months in the PMF group, and mean 78.3 months in the post-PV/ET MF group. As a result of the Log Rank test, no significant difference was observed between the two groups in terms.

Conclusion: The results of this study demonstrated that PMF and post-PV/ET MF patients showed similar demographic, clinical and prognostic features in general. Therefore, patients with ET and PV should be closely monitored for MF development and should be managed as PMF if MF develops.

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Hematology, Transfusion and Cell Therapy
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