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Nam, Kwon, Lee, Nam, Lee, Mun, Seung, Park, Bang, Cho, Shin, and Lee: Vascular Complications in Patients with Essential Thrombocythemia
Original Article

Korean J Hematol 2006;41(3):149-156.

Published online: 19 September 2006

DOI: https://doi.org/10.5045/kjh.2006.41.3.149

Vascular Complications in Patients with Essential Thrombocythemia

Eunmi Nam, M.D.1, Jung Mi Kwon, M.D., Soon Nam Lee, M.D., Seung Hyun Nam, M.D., Kyoung-Eun Lee, M.D., Yeung-Chul Mun, M.D., Chu-Myung Seung, M.D., Se Hoon Park, M.D.1, Soo-Mee Bang, M.D.1, Eun Kyung Cho, M.D.1, Dong Bok Shin, M.D.1, Jae Hoon Lee, M.D.1

Department of Internal Medicine, Ewha Womans University College of Medicine, Seoul, Korea

1Department of Internal Medicine, Gachon University of Medicine and Science Gil Medical Center, Incheon, Korea

Correspondence to：Soon Nam Lee, M.D., Ph.D. Department of Internal Medcine, Ewha Womans University College of Medicine 70, Jongno 6-ga, Jongno-gu, Seoul 110-783, Korea Tel: +82-2-760-5054, Fax: +82-2-762-7756 E-mail: snlee@ewha.ac.kr

Received 18 July 2006       Revised 2 August 2006       Accepted 22 August 2006

Copyright © 2006 Korean Society of Hematology

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background:

Essential thrombocythemia (ET) has a chronic course, but its main clinical features are thrombosis and hemorrhage. We evaluated the clinical features, including the vascular complications in patients with ET, during the disease courses and we determined the predictable risk factors for major vascular complications.

Methods:

From 1991 to 2004, the medical records for 69 patients with ET were retrospectively reviewed for evaluating the clinical features, including the vascular complications, and the predictable risk factors for major vascular complications were analyzed.

Results:

Major vascular thrombotic and hemorrhagic complications were observed in 16 patients (23.2%) and 6 patients (8.7%) at the time of diagnosis, and in 13 (18.8%) and 9 patients (13.0%) during follow-up. The incidence of major vascular thromboses in the older group (age ＞60 years) was higher than that in the younger group (≤60 years) (34.2% vs 9.7%, respectively, P=0.016) at the time of diagnosis. During follow-up, the major vascular thrombosis risk was increased in patients with a previous thrombosis history (37.5% vs 13.2%, respectively, P=0.029) and in patients with 2 or more combined cardiovascular risk factors (44.4% vs 15.0%, respectively, P=0.035). The probability of 10-year survival in patients with thrombo-hemorrhagic complications during the disease course was lower than that in patients without complication (60.5% vs 93.7%, respectively, P=0.046).

Conclusion:

Advanced age, a previous thrombosis history and the combined cardiovascular risk factors were the risk factors for major vascular thrombosis in patients with ET. Prevention of thrombo-hemorrhagic complications is the most important therapeutic goal. Treatment strategies according to risk factors ought to be prospectively investigated.

Keywords: Essential thrombocythemia, Thrombosis, Hemorrhage, Cardiovascular risk factors

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Fig. 1
Survival curves of essential thromcythemia patients. (A) Overall survival of all patients, (B) survival according to major vascular thrombo-hemorrhagic complication.
kjh-41-149f1.tif
Table 1.
Patient characteristics
Age [(years), median and range] 62 (14～91)
Sex [ratio (M/F)] 1:0.68 (41/28)
Hemoglobin [(g/dL), mean±SD] 13.0±2.15
Hematocrit [(%), mean±SD] 39.6±6.93
WBC [(×109/L), mean±SD] 12.96±5.85
Platelet [(×109/L), mean±SD] 1,228±578
Splenomegaly (%) 14/69 (20.3)
Abnormal cytogenetics (%)∗ 3/63 (4.8)

∗Cytogenetic study was missed in 6 patients.

Table 2.
Thrombotic and hemorrhagic vascular complications in essential thrombocythemia patients
  No. of patients %
At time of diagnosis
  Major arterial thrombosis
    Cerebral infarctions 10 14.5
    TIA 1 1.4
    Myocardial infarctions 3 4.3
    Peripheral artery 2 2.9
  Microvascular circulation disturbances
    Dizziness 17 24.6
    Headache 13 18.8
    Erythromelalgia 2 2.9
  Hemorrhage
    Gastrointestinal 1 1.4
    Intramuscular 3 4.3
    Gum bleeding 2 2.9
During follow-up
  Major thrombotic complications
    Cerebral infarctions 3 4.3
    TIA 2 2.9
    Myocardial infarctions 3 4.3
    Angina pectoris 3 4.3
    Splenic infarction 1 1.4
    Peripheral artery 1 1.4
    Deep vein thrombosis 1 1.4
  Hemorrhagic complications
    Intracranial 1 1.4
    Gastrointestinal 4 5.8
    Intramuscular 1 1.4
    Minor bleeding 3 4.3

Abbreviation: TIA, Transient ischemic attack.

Table 3.
Subgroup analysis of the incidence of major thrombotic complications at the time of diagnosis in essential thrombocythemia patients
Category No. of patients of risk No. of patients of event Incidence (%) P value
Age (years)
  ≤60 31 3 9.7 0.016
  ＞60 38 13 9.7
Sex
  Male 41 9 21.9 0.768
  Female 28 7 25.0
No. of cardiovascular risk factors∗
  ＜2 60 13 33.3 0.439
  ≥2 9 3 33.3
Platelet count
  ＜1,000×109/L 26 8 30.8 0.292
  ≥1,000×109/L 41 8 19.5

∗Cardiovascular risk factors including diabetes, hypertension, hyperlipidemia, smoking, obesity.

Table 4.
Subgroup analysis of the incidence of major thrombotic complications during the follow-up in essential thrombocythem patients
Category No. of patients of risk No. of patients of event Incidence (%) P valu
Age (years)
  ≤60 31 6 19.4 0.921
  ＞60 38 7 18.4
Sex
  Male 41 10 24.4 0.768
  Female 28 3 10.7
No. of cardiovascular risk factors∗
  ＜2 60 9 15.0 0.035
  ≥2 9 4 44.4
Initial thrombotic complicatiions
  No 53 7 13.2 0.029
  Yes 16 6 37.5
Treatment
  Hydroxyurea 46 6 13.0 0.082
  Anagrelide 23 7 30.4

∗Cardiovascular risk factors including diabetes, hypertension, hyperlipidemia, smoking, obesity.

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