Contributions
Abstract: PB2312
Type: Publication Only
Background
Thrombosis is one of the most important complication among patients with polycythemia vera (PV) and, in the bibliography, roughly one-fifth of patients with PV are diagnosed with an arterial or venous thrombotic event as a presenting feature. Fatal cardiovascular events contribute to the increased mortality rate among patients with PV and thrombotic events are so inextricably tied to the pathogenesis of PV that they are used in guiding patient risk stratification and treatment recommendations. In addition to several conventional risk factors for thrombosis, clinical data have implicated increased hematocrit and red blood cell adhesiveness, activated platelets, leukocytosis, and elevated JAK2V617F allele burden.
Aims
The goal of our study was to analyze the incidence and the different moment and form of presentation of thrombosis in patients with PV in our centre.
Methods
A retrospective analysis was conducted based on the review of medical records of the adult patients diagnosed of polycythemia vera in our centre from 1986 to 2017. Clinical and biological data were recorded .
Results
A total of 51 patients were analyzed (28 men and 23 women). Mean age at diagnosis was 61 years old (range 28-83). We found a total of 19 patients (37,2%) who suffered a thrombosis before or after PV diagnosis. Among patients with thrombosis before the diagnosis of PV different scenarios have been found:
1) Two patients developed a thrombotic phenomena that led to diagnosis of PV. Both suffered a stroke and one had heterozygous JAK2 mutation while the other one had mutation of exon2-JAK2.
2) Seven patients developed a thrombosis prior to PV diagnosis and without erythrocytosis [4 strokes, 1 acute myocardial infarction (AMI) and 2 pulmonary embolisms]. All of them had heterozygous JAK2 mutation except one that had homozygous mutation.
3) Four patients had a thrombosis before PV diagnosis but after the evidence of erythrocytosis (2 strokes and 2 AMI). Time from thrombosis until hematologist evaluation range from 1 to 29 months (mean 14,5 months). Time from evidence of polycythemia until hematologist evaluation range from 2 to 123 months (mean 43 months). All these patients had heterocygous JAK2 mutation.
On other hand, six patients developed a thrombotic phenomenon after diagnosis (3 strokes , 2 AMI and 1 deep venous thrombosis). Two patients presented homozygous mutation for JAK2 and four heterozygous mutation. Four patients had leukocitosis at diagnosis also. About treatment, three patients were receiving hydroxyurea and two patients phlebotomy when thrombosis occurred, but two male patients had an hematocrit above 45%. Of note, five of these patients were without aspirin prophilaxis when developed the thrombosis .
Conclusion
In our series 37,2% of patients with PV developed thrombosis, especially before the diagnosis. All of them had JAK2-V617F mutation except one who had exon12-JAK2 mutation. Four thrombosis (21%) developed after erythrocytosis was observed but before the patient was evaluated by an hematologist. We wonder if they could have been avoided. After diagnosis, the suspension of aspirin prophylaxis by any reason, seems to be an important factor to develop thrombosis.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Polycythemia vera, Thrombosis
Abstract: PB2312
Type: Publication Only
Background
Thrombosis is one of the most important complication among patients with polycythemia vera (PV) and, in the bibliography, roughly one-fifth of patients with PV are diagnosed with an arterial or venous thrombotic event as a presenting feature. Fatal cardiovascular events contribute to the increased mortality rate among patients with PV and thrombotic events are so inextricably tied to the pathogenesis of PV that they are used in guiding patient risk stratification and treatment recommendations. In addition to several conventional risk factors for thrombosis, clinical data have implicated increased hematocrit and red blood cell adhesiveness, activated platelets, leukocytosis, and elevated JAK2V617F allele burden.
Aims
The goal of our study was to analyze the incidence and the different moment and form of presentation of thrombosis in patients with PV in our centre.
Methods
A retrospective analysis was conducted based on the review of medical records of the adult patients diagnosed of polycythemia vera in our centre from 1986 to 2017. Clinical and biological data were recorded .
Results
A total of 51 patients were analyzed (28 men and 23 women). Mean age at diagnosis was 61 years old (range 28-83). We found a total of 19 patients (37,2%) who suffered a thrombosis before or after PV diagnosis. Among patients with thrombosis before the diagnosis of PV different scenarios have been found:
1) Two patients developed a thrombotic phenomena that led to diagnosis of PV. Both suffered a stroke and one had heterozygous JAK2 mutation while the other one had mutation of exon2-JAK2.
2) Seven patients developed a thrombosis prior to PV diagnosis and without erythrocytosis [4 strokes, 1 acute myocardial infarction (AMI) and 2 pulmonary embolisms]. All of them had heterozygous JAK2 mutation except one that had homozygous mutation.
3) Four patients had a thrombosis before PV diagnosis but after the evidence of erythrocytosis (2 strokes and 2 AMI). Time from thrombosis until hematologist evaluation range from 1 to 29 months (mean 14,5 months). Time from evidence of polycythemia until hematologist evaluation range from 2 to 123 months (mean 43 months). All these patients had heterocygous JAK2 mutation.
On other hand, six patients developed a thrombotic phenomenon after diagnosis (3 strokes , 2 AMI and 1 deep venous thrombosis). Two patients presented homozygous mutation for JAK2 and four heterozygous mutation. Four patients had leukocitosis at diagnosis also. About treatment, three patients were receiving hydroxyurea and two patients phlebotomy when thrombosis occurred, but two male patients had an hematocrit above 45%. Of note, five of these patients were without aspirin prophilaxis when developed the thrombosis .
Conclusion
In our series 37,2% of patients with PV developed thrombosis, especially before the diagnosis. All of them had JAK2-V617F mutation except one who had exon12-JAK2 mutation. Four thrombosis (21%) developed after erythrocytosis was observed but before the patient was evaluated by an hematologist. We wonder if they could have been avoided. After diagnosis, the suspension of aspirin prophylaxis by any reason, seems to be an important factor to develop thrombosis.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Polycythemia vera, Thrombosis