Characteristics and treatment of patients with essential thrombocythemia during the period of 2008–2011
Background: Essential thrombocythemia (ET) is a chronic Ph negative myeloproliferative neoplasm characterized by thrombocytosis, bone marrow megakaryocyte proliferation, JAK 2 V617F mutation in 50–60 % of patients and MPL mutation in 3–5 % of patients. Higher mortality of ET patients is caused by vascular complications, such as thrombosis, microvascular disorders and bleeding events. The goal of treatment is the prevention of thrombosis and haemorrhage. The research was aimed to provide insight into the number of patients and the ways they were treated in the last four years at our clinic. Method: Data from patients diagnosed with ET in the period from 1 January 2008 to 31 December 2011at the Haematology Department of the UMC Ljubljana were collected. The data were analysed in MS Excel. Results: During the period between January 2008 and December 2011 there were 245 new ET patients (35 % males and 65 % females) diagnosed and treated at our clinic. Average age of patients was 65 years. The number of patients with JAK 2 mutation present was 124 (50 %). 180 patients met criteria for ET, while for another 65 the data were insuficient or presence of reactive causes was determined. Patients < 60 years old, who already suffered thrombosis or bleeding events and whose thrombocyte concentration results exceeded 1500x10/9/L were grouped as patients with clear criteria for ET and high risk for thrombosis. The number of patients was 132; 49 patients were qualified for the group with minor risk for complications. Risk factors for cardiovascular disease, which causes upward risk for thrombosis, were determined in 47 % of all patients. The majority of patients received treatment with low-dose aspirin. Cytoreductive therapy with hydroxyurea and anagrelide. A few patients received treatment with a combination of hydroxyurea and anagrelide. Only some patients received interferon alpha or busulfane. One patient presented with transition into primary myelofibrosis and another one with transition into acute leukemia. Conclusions: In the analyzed group of patients, we have established the incidence of the disease in all age groups . The disease affects women at a higher rate. The results comply with so far known facts and research. The presence of JAK2 mutation is consistent with known statistic facts. The proportion of reactive trombocytoses reffered to as ET is outstanding (26.5 %). The fact that the shares of patients treated with hydroxyurea and anagrelide are practically equal is unusual. According to the expectations and guidelines it would be expected that anagrelide is introduced as a second-line treatment. In our four-year observation period, transitions to primary myelofibrosis and acute leukemia were considerably low..
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2012 |
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| Erschienen: |
2012 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:81 |
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| Enthalten in: |
Zdravniški Vestnik - 81(2012), SUPL II |
| Sprache: |
Englisch ; Slowenisch |
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| Beteiligte Personen: |
Karla Rener [VerfasserIn] |
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| Links: |
doaj.org [kostenfrei] |
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| Themen: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
DOAJ003420132 |
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| 520 | |a Background: Essential thrombocythemia (ET) is a chronic Ph negative myeloproliferative neoplasm characterized by thrombocytosis, bone marrow megakaryocyte proliferation, JAK 2 V617F mutation in 50–60 % of patients and MPL mutation in 3–5 % of patients. Higher mortality of ET patients is caused by vascular complications, such as thrombosis, microvascular disorders and bleeding events. The goal of treatment is the prevention of thrombosis and haemorrhage. The research was aimed to provide insight into the number of patients and the ways they were treated in the last four years at our clinic. Method: Data from patients diagnosed with ET in the period from 1 January 2008 to 31 December 2011at the Haematology Department of the UMC Ljubljana were collected. The data were analysed in MS Excel. Results: During the period between January 2008 and December 2011 there were 245 new ET patients (35 % males and 65 % females) diagnosed and treated at our clinic. Average age of patients was 65 years. The number of patients with JAK 2 mutation present was 124 (50 %). 180 patients met criteria for ET, while for another 65 the data were insuficient or presence of reactive causes was determined. Patients < 60 years old, who already suffered thrombosis or bleeding events and whose thrombocyte concentration results exceeded 1500x10/9/L were grouped as patients with clear criteria for ET and high risk for thrombosis. The number of patients was 132; 49 patients were qualified for the group with minor risk for complications. Risk factors for cardiovascular disease, which causes upward risk for thrombosis, were determined in 47 % of all patients. The majority of patients received treatment with low-dose aspirin. Cytoreductive therapy with hydroxyurea and anagrelide. A few patients received treatment with a combination of hydroxyurea and anagrelide. Only some patients received interferon alpha or busulfane. One patient presented with transition into primary myelofibrosis and another one with transition into acute leukemia. Conclusions: In the analyzed group of patients, we have established the incidence of the disease in all age groups . The disease affects women at a higher rate. The results comply with so far known facts and research. The presence of JAK2 mutation is consistent with known statistic facts. The proportion of reactive trombocytoses reffered to as ET is outstanding (26.5 %). The fact that the shares of patients treated with hydroxyurea and anagrelide are practically equal is unusual. According to the expectations and guidelines it would be expected that anagrelide is introduced as a second-line treatment. In our four-year observation period, transitions to primary myelofibrosis and acute leukemia were considerably low. | ||
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