The Proportion, Clinical characteristics, complications, treatment pattern & outcomes of Ph-negative Classic Myeloproliferative Neoplasms at TASH: A Hospital based retrospective Cross-Sectional study.
| dc.contributor.advisor | Abdela,Fozia (MD, Ass.Prof.) | |
| dc.contributor.author | Yirgu,Dahlak Teshome | |
| dc.date.accessioned | 2025-08-12T20:04:30Z | |
| dc.date.available | 2025-08-12T20:04:30Z | |
| dc.date.issued | 2024-03 | |
| dc.description.abstract | Background Haematological malignancies are among non-communicable diseases that are becoming increasingly more common both globally and in our country, Ethiopia. Out of these Philadelphia-negative classic myeloproliferative neoplasms, affecting the elderly population group of the society is seen in increased frequency in outpatient department. This is due to progressively rising global population age and life expectancy, so it is becoming an important public health problem worldwide. Investigations done with a small number of African countries indicated a gradually increasing frequency of philadelphia-negative MPNs in the continent. In growing country, there are few studies that truly define the clinical feature of these patients. Especially in Ethiopia there is no data regarding Ph negative classic MPNs. This will aid to estimate the characteristics, treatment pattern & outcomes based on a local data of patients with diagnosed Philadelphia-negative classic MPNs. Objective: The main objective of the study is to measure the clinical characteristics, complications, treatment pattern & outcomes of Ph negative classic MPN patients. Methods: A single cantered hospital based retrospective cross sectional study was conducted at haematology follow up clinic from January 1, 2019 to December 31, 2023; data was collected and analyzed with IBM SPSS version 27. Result: A total of 151 study participants with the age of 18 & above were included in this study. 57 %( 86/151) of study participants are male & 43% are females, with a male to female ratio of 1.3:1. 58.3% of PV patients were male, 56.7% of ET patients were females. Similarly 72.2% of PMF cases were male. PV was the most common (68%) with a median age at diagnosis of 56 years, followed by ET (20%, median age 55.5 years) and Primary Myelofibrosis (12%, median age 60 years). Female patients had mostly a history of ET; a higher number of male patients had a history of PV and PMF. Among PV cases, the main presenting symptoms were headache/vertigo (37%) followed by fatigue (31.4%), early satiety/ LUQ pain (31%) & splenomegaly was found in 28%. In ET, the main presenting symptoms were fatigue (36.8%), headache, joint pain, and splenomegaly being the other common presenting symptoms Among the PMF patients’, the main presenting symptoms were symptoms of anaemia (40%), fatigue (38%), constitutional symptoms (35%), early satiety/ LUQ pain (35%) & Splenomegaly (72%). JAK2V617 mutation was positive in 92%, 54%, and 44.4% of PV, ET and PMF patients, respectively. The frequency of thrombotic events were high in PV patients compared to other subtypes (PV=33%, ET=26% and PMF=5.6%) and venous thrombosis being higher than arterial. In contrast to other subtypes, bleeding was prevalent in PMF patients (PMF=11%). Thrombotic complication was high in high risk PV & ET patients, while Jak 2 mutation status didn’t affect thrombosis risk. Phlebotomy, ASA and hydroxyurea, Thalidomide/prednisolone and transfusion support were the treatment options used. The five year survival rate of Ph –ve MPNs with median diagnosis of 27months were 81%, which was higher for ET (95%), PV=84% and low for PMF (median survival rate is 4 years). Conclusion: This study showed that, PV is a more prevalent ph negative MPN than ET and PMF. Thrombosis is an important complication in these patients, especially in PV patients occurring in ~1/3rd of the cases. Based on our findings we emphasize that that Ph negative MPNs should be considered & proper evaluation should follow in patient with unexplained abnormalities in hematologic parameters. | |
| dc.identifier.uri | https://etd.aau.edu.et/handle/123456789/6505 | |
| dc.language.iso | en_US | |
| dc.publisher | Addis Ababa University | |
| dc.subject | Haematological Malignancies | |
| dc.title | The Proportion, Clinical characteristics, complications, treatment pattern & outcomes of Ph-negative Classic Myeloproliferative Neoplasms at TASH: A Hospital based retrospective Cross-Sectional study. | |
| dc.type | Thesis |