Profile of Philadelphia Chromosome Negative (Ph-) Myeloproliferative Neoplasm with Special Emphasis on Vascular Thrombotic Events and the Response to Cytoreductive Therapy in Polycythemia Vera and Essential Thrombocythemia Patients: A Single Center Study from Kerala.
Thrombotic events are a major morbidity among Philadelphia chromosome-negative myeloproliferative neoplasm (Ph-MPN) patients. There is a lack of data from Kerala regarding the profile of Ph-MPN and the prevalence of thrombosis among these patients.
To study the clinical profile, driver mutations, incidence of thrombotic events among Ph-MPN patients, and the response to hydroxyurea therapy.
We reviewed the medical records of 84 Ph-MPN patients who were on follow-up from April 2019 to June 2023 in a tertiary care hospital in Kerala.
There were 48 polycythemia vera (PV), 16 essential thrombocythemia (ET), 14 primary myelofibrosis (PMF), and six unclassifiable MPN (MPN-u) patients. The incidence of Janus kinase 2 (JAK2) mutation was 96, 62.5, and 79% among PV, ET, and PMF patients, respectively. The incidence of calreticulin (CALR) mutation was 37.5 and 21% among ET and PMF patients, respectively. The incidence of thrombotic events was 23/48 (48%), 7/16 (43.5%), and 6/14 (42.8%) among PV, ET, and PMF patients, respectively. All ET and PMF patients with thrombotic events were JAK2V617F-mutated. Eighty-seven percent of the evaluable patients on hydroxyurea for PV achieved freedom from therapeutic phlebotomies. ET patients who were on hydroxyurea achieved a median platelet count of 4.3 lakhs/µL (3.16-6.08).
There is a higher incidence of thrombosis among Ph-MPN patients from Kerala, which needs to be ascertained in a population-based study. JAK2V617F mutation is the major determinant of thrombotic episodes in ET and PMF. Hydroxyurea is an effective cytoreductive therapy in PV and ET.
To study the clinical profile, driver mutations, incidence of thrombotic events among Ph-MPN patients, and the response to hydroxyurea therapy.
We reviewed the medical records of 84 Ph-MPN patients who were on follow-up from April 2019 to June 2023 in a tertiary care hospital in Kerala.
There were 48 polycythemia vera (PV), 16 essential thrombocythemia (ET), 14 primary myelofibrosis (PMF), and six unclassifiable MPN (MPN-u) patients. The incidence of Janus kinase 2 (JAK2) mutation was 96, 62.5, and 79% among PV, ET, and PMF patients, respectively. The incidence of calreticulin (CALR) mutation was 37.5 and 21% among ET and PMF patients, respectively. The incidence of thrombotic events was 23/48 (48%), 7/16 (43.5%), and 6/14 (42.8%) among PV, ET, and PMF patients, respectively. All ET and PMF patients with thrombotic events were JAK2V617F-mutated. Eighty-seven percent of the evaluable patients on hydroxyurea for PV achieved freedom from therapeutic phlebotomies. ET patients who were on hydroxyurea achieved a median platelet count of 4.3 lakhs/µL (3.16-6.08).
There is a higher incidence of thrombosis among Ph-MPN patients from Kerala, which needs to be ascertained in a population-based study. JAK2V617F mutation is the major determinant of thrombotic episodes in ET and PMF. Hydroxyurea is an effective cytoreductive therapy in PV and ET.