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
C2 - 22075289
AN - SCOPUS:80855148205
SN - 0953-6205
VL - 22
SP - 607
EP - 610
JO - European Journal of Internal Medicine
JF - European Journal of Internal Medicine
IS - 6
ER -