MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA

Essential thrombocytosis (ET) is clonal chronic myeloproliferative disorder which originates from abnormality of a multipotent hematopoietic stem cell.It is characterized by an increased platelet count, megakaryocytic hyperplasia and by hemorrhagic or thrombotic tendency. Symptoms and signs may incl...

Full description

Bibliographic Details
Main Authors: Lana Macukanovic-Golubovic, Irena Cojbasic
Format: Article
Language:English
Published: University in Nis, Faculty of Medicine 2008-10-01
Series:Acta Medica Medianae
Subjects:
Online Access:http://publisher.medfak.ni.ac.rs/2008-html/3-broj/MODALITY%20OF%20TREATMENT....pdf
_version_ 1818253888694779904
author Lana Macukanovic-Golubovic
Irena Cojbasic
author_facet Lana Macukanovic-Golubovic
Irena Cojbasic
author_sort Lana Macukanovic-Golubovic
collection DOAJ
description Essential thrombocytosis (ET) is clonal chronic myeloproliferative disorder which originates from abnormality of a multipotent hematopoietic stem cell.It is characterized by an increased platelet count, megakaryocytic hyperplasia and by hemorrhagic or thrombotic tendency. Symptoms and signs may include weakness, headaches, paresthesias, bleeding, splenomegaly, and digital ischemia. ET patients showed equal or slightly shorter survival than age- and sex-matched healthy population. Major causes of death were thrombotic and hemorrhagic complications or malignant progression due to both the natural history of the disease and, possibly, the use of chemotherapeutic agents.Diagnostic criteria for essential thrombocythemia were proposed in 2005 by the PVSG and demand diagnosis of exclusion.Myelosuppressive therapy to lower the platelet count usually consists of hydroxyurea, interferon alpha or anagrelide. Hydroxyurea is the most commonly used treatment, because of its efficacy, low cost and rare acute toxicity. Interferon alpha is a biological response modifier. It is not known to be teratogenic and does not cross the placenta, and is often the treatment of choice during pregnancy. Anagrelid suppresses bone marrow megakaryocytes by interfering with the maturation process and decreasing platelet production without affecting other blood cell lines. Low-dose aspirin may be used to control microvascular symptoms.Recommendations for management of patients with essential thrombocythemia were given by ASH. From a treatment standpoint, hydroxyurea is now confirmed to be the drug of choice for high-risk patients with essential thrombocythemia. Interferon alpha and anagrelide are reasonable second-line agents. Low-risk patients should receive low-dose aspirin alone. For the intermediate-risk patients, a consensus could not be reached on a recommendation for platelet-lowering treatment.
first_indexed 2024-12-12T16:47:14Z
format Article
id doaj.art-311711db670048009bb54fc565d77ead
institution Directory Open Access Journal
issn 0365-4478
language English
last_indexed 2024-12-12T16:47:14Z
publishDate 2008-10-01
publisher University in Nis, Faculty of Medicine
record_format Article
series Acta Medica Medianae
spelling doaj.art-311711db670048009bb54fc565d77ead2022-12-22T00:18:27ZengUniversity in Nis, Faculty of MedicineActa Medica Medianae0365-44782008-10-014735155MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIALana Macukanovic-GolubovicIrena CojbasicEssential thrombocytosis (ET) is clonal chronic myeloproliferative disorder which originates from abnormality of a multipotent hematopoietic stem cell.It is characterized by an increased platelet count, megakaryocytic hyperplasia and by hemorrhagic or thrombotic tendency. Symptoms and signs may include weakness, headaches, paresthesias, bleeding, splenomegaly, and digital ischemia. ET patients showed equal or slightly shorter survival than age- and sex-matched healthy population. Major causes of death were thrombotic and hemorrhagic complications or malignant progression due to both the natural history of the disease and, possibly, the use of chemotherapeutic agents.Diagnostic criteria for essential thrombocythemia were proposed in 2005 by the PVSG and demand diagnosis of exclusion.Myelosuppressive therapy to lower the platelet count usually consists of hydroxyurea, interferon alpha or anagrelide. Hydroxyurea is the most commonly used treatment, because of its efficacy, low cost and rare acute toxicity. Interferon alpha is a biological response modifier. It is not known to be teratogenic and does not cross the placenta, and is often the treatment of choice during pregnancy. Anagrelid suppresses bone marrow megakaryocytes by interfering with the maturation process and decreasing platelet production without affecting other blood cell lines. Low-dose aspirin may be used to control microvascular symptoms.Recommendations for management of patients with essential thrombocythemia were given by ASH. From a treatment standpoint, hydroxyurea is now confirmed to be the drug of choice for high-risk patients with essential thrombocythemia. Interferon alpha and anagrelide are reasonable second-line agents. Low-risk patients should receive low-dose aspirin alone. For the intermediate-risk patients, a consensus could not be reached on a recommendation for platelet-lowering treatment.http://publisher.medfak.ni.ac.rs/2008-html/3-broj/MODALITY%20OF%20TREATMENT....pdfessential thrombocythemiarecommendationtreatment
spellingShingle Lana Macukanovic-Golubovic
Irena Cojbasic
MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA
Acta Medica Medianae
essential thrombocythemia
recommendation
treatment
title MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA
title_full MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA
title_fullStr MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA
title_full_unstemmed MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA
title_short MODALITY OF TREATMENT IN ESSENTIAL THROMBOCYTHEMIA
title_sort modality of treatment in essential thrombocythemia
topic essential thrombocythemia
recommendation
treatment
url http://publisher.medfak.ni.ac.rs/2008-html/3-broj/MODALITY%20OF%20TREATMENT....pdf
work_keys_str_mv AT lanamacukanovicgolubovic modalityoftreatmentinessentialthrombocythemia
AT irenacojbasic modalityoftreatmentinessentialthrombocythemia