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SPLANCHNIC THROMBOSES IN MYELOPROLIFERATIVE DISORDERS - EXPERIENCE OF THE HEMATOLOGY CENTER OF FUNDENI CLINICAL INSTITUTE
Author(s): ,
Iulia Ursuleac
Affiliations:
Hematology,Fundeni Clinical Institute,Bucharest,Romania;'Carol Davila' University of Medicine and Pharmacy,Bucharest,Romania
,
Roxana Dragan
Affiliations:
Hematology,Fundeni Clinical Institute,Bucharest,Romania
,
Alexandru Bardas
Affiliations:
Hematology,Fundeni Clinical Institute,Bucharest,Romania
,
Cristina Constantin
Affiliations:
Hematology,Fundeni Clinical Institute,Bucharest,Romania
,
Camelia Dobrea
Affiliations:
Hematology,Fundeni Clinical Institute,Bucharest,Romania;'Carol Davila' University of Medicine and Pharmacy,Bucharest,Romania
Daniel Coriu
Affiliations:
Hematology,Fundeni Clinical Institute,Bucharest,Romania;'Carol Davila' University of Medicine and Pharmacy,Bucharest,Romania
(Abstract release date: 05/17/18) EHA Library. Ursuleac I. 06/14/18; 216556; PB2299
Iulia Ursuleac
Iulia Ursuleac
Contributions
Abstract

Abstract: PB2299

Type: Publication Only

Background

Myeloproliferative disorders are known to cause splanchnic thromboses. Risk factors for thromboses in myeloproliferative neoplasms are: age > 60years, previous thromboses, JAK2V617F mutation positivity, associated thrombophilic status, pancytosis, and classical risk factors (obesity, dislipidemia, diabetes, smoking, use of oral contraceptives).

Aims

Evaluating risk factors for splanchnic thromboses in a group of pacients with myeloproliferative neoplasms, unicentric analysis over 7 years (2010-2017) in Fundeni Hematology Clinic.

Methods
Clinical and epidemiological retrospective study of 20 cases of patients diagnosed with myeloproliferative neoplasms and visceral thromboses. 

Results

Clinical and epidemiological data: 20 patients diagnosed with myeloproliferative neoplasms and visceral thromboses (14 women, 6 men) of median age 34.5 years (20-55 years):  4 cases of polycythemia vera, 7 essential thrombocytemia, 7 myelofibrosis, 2 unclassified myeloproliferative disorder. In 18 cases JAK2V617F mutation was present, 1 case negative and 1 was not tested. 10 patients with suprahepatic vein thrombosis, the majority positive for JAK2V617F mutation (9 cases). Portal vein thrombosis was found in 3 cases, all of them positive for JAK2V617F mutation, and associations of suprahepatic vein thrombosis with portal vein thrombosis was found in 7 cases.  Pancytosis (Ht>44%, Le>11.000/mmc, Tr>450.000/mmc) was found in 5 cases, association of Ht>44% and Tr>450.000/mmc in 4 cases. Association between Ht>44% and leucocytosis over 11.000/mmc was found in 1 case. Normal blood cell counts values were detected in 4 cases. We identified the following as additional risk factors for thrombosis: smoking (6 cases), dislipidemia (3), use of oral contraceptives (2), thrombophilia (5). Two or more risk factors were present in 5 cases. Anticoagulant therapy was associated with cytoreductive therapy in 18  cases and 2 patients required only anticoagulant therapy. Recurrent thromboses were found in 2 cases. Death caused by progression of hematological disease occurred in 3 cases; all other patiens are hematologically stable under specific treatment. Due to progressive hepatic disease, 3 patients received liver transplants and 2 patients underwent transjugular intrahepatic portosystemic shunt.

Conclusion

Presence of splanchnic thrombosis is highly suggestive for the diagnosis of myeloproliferative neoplasms - 12 of our patients were diagnosed simultaneously for both pathologies, and in 8 patients the thrombotic episode occurred after the diagnosis of hematological disease. In the studied group of 20 cases, JAK2V617F mutation was identified in 18 cases. Most frequent myeloproliferative neoplasms were essential thrombocytemia and myelofibrosis, 7 cases each. Cytoreductive therapy associated with anticoagulant therapy represented therapeutical option for 18 cases. The evolution of thrombosis depends on the therapeutical response of the hematological disorder and also on the possibility for correcting the associated risk factors.

Session topic: 16. Myeloproliferative neoplasms - Clinical

Keyword(s): Myeloproliferative disorder, Risk factor, Thrombosis

Abstract: PB2299

Type: Publication Only

Background

Myeloproliferative disorders are known to cause splanchnic thromboses. Risk factors for thromboses in myeloproliferative neoplasms are: age > 60years, previous thromboses, JAK2V617F mutation positivity, associated thrombophilic status, pancytosis, and classical risk factors (obesity, dislipidemia, diabetes, smoking, use of oral contraceptives).

Aims

Evaluating risk factors for splanchnic thromboses in a group of pacients with myeloproliferative neoplasms, unicentric analysis over 7 years (2010-2017) in Fundeni Hematology Clinic.

Methods
Clinical and epidemiological retrospective study of 20 cases of patients diagnosed with myeloproliferative neoplasms and visceral thromboses. 

Results

Clinical and epidemiological data: 20 patients diagnosed with myeloproliferative neoplasms and visceral thromboses (14 women, 6 men) of median age 34.5 years (20-55 years):  4 cases of polycythemia vera, 7 essential thrombocytemia, 7 myelofibrosis, 2 unclassified myeloproliferative disorder. In 18 cases JAK2V617F mutation was present, 1 case negative and 1 was not tested. 10 patients with suprahepatic vein thrombosis, the majority positive for JAK2V617F mutation (9 cases). Portal vein thrombosis was found in 3 cases, all of them positive for JAK2V617F mutation, and associations of suprahepatic vein thrombosis with portal vein thrombosis was found in 7 cases.  Pancytosis (Ht>44%, Le>11.000/mmc, Tr>450.000/mmc) was found in 5 cases, association of Ht>44% and Tr>450.000/mmc in 4 cases. Association between Ht>44% and leucocytosis over 11.000/mmc was found in 1 case. Normal blood cell counts values were detected in 4 cases. We identified the following as additional risk factors for thrombosis: smoking (6 cases), dislipidemia (3), use of oral contraceptives (2), thrombophilia (5). Two or more risk factors were present in 5 cases. Anticoagulant therapy was associated with cytoreductive therapy in 18  cases and 2 patients required only anticoagulant therapy. Recurrent thromboses were found in 2 cases. Death caused by progression of hematological disease occurred in 3 cases; all other patiens are hematologically stable under specific treatment. Due to progressive hepatic disease, 3 patients received liver transplants and 2 patients underwent transjugular intrahepatic portosystemic shunt.

Conclusion

Presence of splanchnic thrombosis is highly suggestive for the diagnosis of myeloproliferative neoplasms - 12 of our patients were diagnosed simultaneously for both pathologies, and in 8 patients the thrombotic episode occurred after the diagnosis of hematological disease. In the studied group of 20 cases, JAK2V617F mutation was identified in 18 cases. Most frequent myeloproliferative neoplasms were essential thrombocytemia and myelofibrosis, 7 cases each. Cytoreductive therapy associated with anticoagulant therapy represented therapeutical option for 18 cases. The evolution of thrombosis depends on the therapeutical response of the hematological disorder and also on the possibility for correcting the associated risk factors.

Session topic: 16. Myeloproliferative neoplasms - Clinical

Keyword(s): Myeloproliferative disorder, Risk factor, Thrombosis

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