The Journal of the Association of Physicians of India · 2026
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.
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Prabhu Raghuveer Santhakumara, Nameera Rahmathullah Sulaimankutty, Thangal K Sayyid Muhammed Lukhmanulhakkim, Nair Priyanka R
Аннотация
INTRODUCTION: 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.
AIMS: To study the clinical profile, driver mutations, incidence of thrombotic events among Ph-MPN patients, and the response to hydroxyurea therapy.
METHODS: 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.
RESULTS: 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).
CONCLUSION: 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.
AIMS: To study the clinical profile, driver mutations, incidence of thrombotic events among Ph-MPN patients, and the response to hydroxyurea therapy.
METHODS: 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.
RESULTS: 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).
CONCLUSION: 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.