Risk factors for pulmonary hypertension in patients with β thalassemia intermedia

Mehran Karimi, Khaled M. Musallam, Maria Domenica Cappellini, Shahina Daar, Amal El-Beshlawy, Khawla Belhoul, Mohamed Salaheldin Saned, Sally Temraz, Suzanne Koussa, Ali T. Taher

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Background: Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI). Methods: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I. Results: The mean age of Group I patients at development of PHT was 37.3 ± 10.6 years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≤ 300 × 10 6/l (2.59-times) or a previous history of thromboembolic events (3.69-times). Conclusion: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.

Original languageEnglish
Pages (from-to)607-610
Number of pages4
JournalEuropean Journal of Internal Medicine
Volume22
Issue number6
DOIs
Publication statusPublished - Dec 2011

Fingerprint

beta-Thalassemia
Pulmonary Hypertension
Erythrocyte Count
Hydroxyurea
Iron
Chelation Therapy
Thromboembolism
Cost-Benefit Analysis
Echocardiography
Age Groups
Demography
Clinical Trials
Safety

Keywords

  • Hemolysis
  • Hypercoagulability
  • Pulmonary hypertension
  • Risk factors
  • Thalassemia intermedia
  • Thromboembolism

ASJC Scopus subject areas

  • Internal Medicine

Cite this

Karimi, M., Musallam, K. M., Cappellini, M. D., Daar, S., El-Beshlawy, A., Belhoul, K., ... Taher, A. T. (2011). Risk factors for pulmonary hypertension in patients with β thalassemia intermedia. European Journal of Internal Medicine, 22(6), 607-610. https://doi.org/10.1016/j.ejim.2011.05.013

Risk factors for pulmonary hypertension in patients with β thalassemia intermedia. / Karimi, Mehran; Musallam, Khaled M.; Cappellini, Maria Domenica; Daar, Shahina; El-Beshlawy, Amal; Belhoul, Khawla; Saned, Mohamed Salaheldin; Temraz, Sally; Koussa, Suzanne; Taher, Ali T.

In: European Journal of Internal Medicine, Vol. 22, No. 6, 12.2011, p. 607-610.

Research output: Contribution to journalArticle

Karimi, M, Musallam, KM, Cappellini, MD, Daar, S, El-Beshlawy, A, Belhoul, K, Saned, MS, Temraz, S, Koussa, S & Taher, AT 2011, 'Risk factors for pulmonary hypertension in patients with β thalassemia intermedia', European Journal of Internal Medicine, vol. 22, no. 6, pp. 607-610. https://doi.org/10.1016/j.ejim.2011.05.013
Karimi, Mehran ; Musallam, Khaled M. ; Cappellini, Maria Domenica ; Daar, Shahina ; El-Beshlawy, Amal ; Belhoul, Khawla ; Saned, Mohamed Salaheldin ; Temraz, Sally ; Koussa, Suzanne ; Taher, Ali T. / Risk factors for pulmonary hypertension in patients with β thalassemia intermedia. In: European Journal of Internal Medicine. 2011 ; Vol. 22, No. 6. pp. 607-610.
@article{25f799c1a5f841c9baeb63ffabfa9f03,
title = "Risk factors for pulmonary hypertension in patients with β thalassemia intermedia",
abstract = "Background: Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI). Methods: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I. Results: The mean age of Group I patients at development of PHT was 37.3 ± 10.6 years; with 44{\%} being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≤ 300 × 10 6/l (2.59-times) or a previous history of thromboembolic events (3.69-times). Conclusion: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.",
keywords = "Hemolysis, Hypercoagulability, Pulmonary hypertension, Risk factors, Thalassemia intermedia, Thromboembolism",
author = "Mehran Karimi and Musallam, {Khaled M.} and Cappellini, {Maria Domenica} and Shahina Daar and Amal El-Beshlawy and Khawla Belhoul and Saned, {Mohamed Salaheldin} and Sally Temraz and Suzanne Koussa and Taher, {Ali T.}",
year = "2011",
month = "12",
doi = "10.1016/j.ejim.2011.05.013",
language = "English",
volume = "22",
pages = "607--610",
journal = "European Journal of Internal Medicine",
issn = "0953-6205",
publisher = "Elsevier",
number = "6",

}

TY - JOUR

T1 - Risk factors for pulmonary hypertension in patients with β thalassemia intermedia

AU - Karimi, Mehran

AU - Musallam, Khaled M.

AU - Cappellini, Maria Domenica

AU - Daar, Shahina

AU - El-Beshlawy, Amal

AU - Belhoul, Khawla

AU - Saned, Mohamed Salaheldin

AU - Temraz, Sally

AU - Koussa, Suzanne

AU - Taher, Ali T.

PY - 2011/12

Y1 - 2011/12

N2 - Background: Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI). Methods: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I. Results: The mean age of Group I patients at development of PHT was 37.3 ± 10.6 years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≤ 300 × 10 6/l (2.59-times) or a previous history of thromboembolic events (3.69-times). Conclusion: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.

AB - Background: Pulmonary hypertension (PHT) is a common yet poorly understood complication of β thalassemia intermedia (TI). Methods: We herein evaluated risk factors for PHT in TI, through comparing 64 TI patients with evidence of PHT by symptomatology and echocardiography (Group I) to age- and sex-matched TI patients without PHT (Group II). Retrieved data included demographics, laboratory parameters, clinical characteristics, and received treatments that may influence PHT development; and reflected the period prior to PHT occurrence in Group I. Results: The mean age of Group I patients at development of PHT was 37.3 ± 10.6 years; with 44% being males. Among studied parameters, Group I patients were more likely to be splenectomized (4.9-times), transfusion-naive (3.5-times); hydroxyurea-naive (2.6-times), or iron chelation-naive (2.3-times); and have nucleated red blood cell count ≤ 300 × 10 6/l (2.59-times) or a previous history of thromboembolic events (3.69-times). Conclusion: TI patients who eventually develop PHT may be identified early on by being splenectomized, having high nucleated red blood cell counts and a previous history of thromboembolism. Prospective clinical trials that evaluate the efficacy, safety, and cost effectiveness of transfusion, iron chelation, and hydroxyurea therapy in preventing PHT in TI are invited.

KW - Hemolysis

KW - Hypercoagulability

KW - Pulmonary hypertension

KW - Risk factors

KW - Thalassemia intermedia

KW - Thromboembolism

UR - http://www.scopus.com/inward/record.url?scp=80855148205&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=80855148205&partnerID=8YFLogxK

U2 - 10.1016/j.ejim.2011.05.013

DO - 10.1016/j.ejim.2011.05.013

M3 - Article

VL - 22

SP - 607

EP - 610

JO - European Journal of Internal Medicine

JF - European Journal of Internal Medicine

SN - 0953-6205

IS - 6

ER -