Prevalence and risk factors for pulmonary hypertension in patients with hemoglobin E/β-thalassemia disease

European Journal of Haematology

Volumn 92, Issue 4, 2014, Pages 346-353

  Atichartakarn, Vichai ^a   Chuncharunee, Suporn ^a   Archararit, Napaporn ^a   Udomsubpayakul, Umaporn ^a   Lee, Ratchanee ^a   Tunhasiriwet, Anusith ^a   Aryurachai, Katcharin ^a  

^a MAHIDOL UNIVERSITY   (Thailand)

Author keywords

Asplenia; Hemolysis; Pulmonary hypertension; Thalassemia

Indexed keywords

BIOLOGICAL MARKER; C REACTIVE PROTEIN; DEFERIPRONE; DEFEROXAMINE; HEMOGLOBIN E; VASCULAR CELL ADHESION MOLECULE 1;

ADULT; ARTICLE; ASPLENIA; BETA THALASSEMIA; BLOOD EXAMINATION; FEMALE; HEMOLYSIS; HUMAN; LABORATORY TEST; MAJOR CLINICAL STUDY; MALE; OUTPATIENT; PREVALENCE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RISK FACTOR; SEROLOGY; SPLENECTOMY; THORAX RADIOGRAPHY; TRANSTHORACIC ECHOCARDIOGRAPHY; BETA-THALASSEMIA; COMPLICATION; GENETICS; HYPERTENSION, PULMONARY; RISK; YOUNG ADULT;

ADULT; BETA-THALASSEMIA; BIOLOGICAL MARKERS; FEMALE; HEMOGLOBIN E; HUMANS; HYPERTENSION, PULMONARY; MALE; ODDS RATIO; PREVALENCE; RISK FACTORS; YOUNG ADULT;

EID: 84896548170     PISSN: 09024441     EISSN: 16000609     Source Type: Journal    
DOI: 10.1111/ejh.12242     Document Type: Article

References (34)

1. Gladwin MT, Sachdev V, Jison ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004;350:886-95.
2. Farmakis D, Aessopos A. Pulmonary hypertension associated with hemoglobinopathies. Prevalent but overlooked. Circulation 2011;123:1227-32.
3. World Health Organization. Monogenic diseases. http://www.who.int/genomics/public/geneticdiseases/en/index2.html#SCA.
4. Lukens JN. The thalassemias and related disorders: quantitative disorders of hemoglobin synthesis. In: Lee GR, Foerster J, Lukens J, Paraskevas F, Greer JP, Rodgers GM, eds. Wintrobe's Clinical Hematology, 10th edn. Baltimore: Williams & Wilkins; 1999:1405-48.
5. Castro O, Hoque M, Brown BD. Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. Blood 2002;101:1257-61.
6. Phrommintikul A, Sukonthasarn A, Kanjanavanit R, Nawarawong W. Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia. Heart 2006;92:1467-72.
7. Lang RM, Bierig M, Devereux RB, et al. Recommendations for chamber quantification. J Am Soc Echocardiogr 2005;18:1440-63.
8. Guazzi M, Borlaug BA. Pulmonary hypertension due to left heart disease. Circulation 2012;126:975-90.
9. Rich JD, Shah SJ, Swamy RS, Kamp A, Rich S. Inaccuracy of doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice. Chest 2011;139:988-93.
10. Machado RF, Gladwin MT. Pulmonary hypertension in hemolytic disorders. Pulmonary vascular disease: the global perspective. Chest 2010;137(Suppl):30S-8S.
11. Rich S. Pulmonary hypertension. In: Braunwald E, Zipes DP, Libby P, eds. Heart Disease: A Textbook of Cardiovascular Medicine. Philadelphia: WB Saunders; 2001:1908-35.
12. Atichartakarn V, Chuncharunee S, Yamwong S, Yingchoncharoen T, Jongjirasiri S, Archararit N, Udomsubpayakul U. Pulmonary artery pressure correlates directly with spleen volume in non-splenectomized hemoglobin E/β-thalassemia patients. Acta Haematol 2013;130:172-5.
13. Atichartakarn V, Likittanasombat K, Chuncharunee S, Chandanamattha P, Worapongpaiboon S, Angchaisuksiri P, Aryurachai K. Pulmonary arterial hypertension in previously splenectomized patients with beta-thalassemic disorders. Int J Hematol 2003;78:139-45.
14. Morris CR, Kim HY, Trachtenberg F, et al. Risk factors and mortality associated with an elevated tricuspid regurgitant jet velocity measured by Doppler-echocardiography in thalassemia: a Thalassemia Clinical Research Network report. Blood 2011;118:3794-802.
15. Premawardhena A, Fisher CA, Olivieri NF, et al. Haemoglobin E beta thalassaemia in Sri Lanka. Lancet 2005;366:1467-70.
16. Rother RP, Bell L, Hillmen P, Gladwin MT. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA 2005;293:1653-62.
17. Muller WA. Leukocyte-endothelial cell interactions in the inflammatory response. Lab Invest 2002;82:521-33.
18. Setty BNY, Stuart MJ, Dampier C, Brodecki D, Allen JL. Hypoxaemia in sickle cell disease: biomarker modulation and relevance to pathophysiology. Lancet 2003;362:1450-5.
19. Haraldsen G, Kvale D, Lien B, Farstad IN, Brandtzaeg P. Cytokine-regulated expression of E-selectin, intercellular adhesion molecule-1, and vascular adhesion molecule in human microvascular endothelial cells. J Immunol 1996;156:2558-65.
20. Esmon CT. The interactions between inflammation and coagulation. Br J Haematol 2005;131:417-30.
21. Atichartakarn V, Angchaisuksiri P, Aryurachai K, Onpun S, Chuncharunee S, Thakkinstian A, Atamasirikul K. Relationship between hypercoagulable state and erythrocyte phosphatidylserine exposure in splenectomized haemoglobin E/β-thalassaemic patients. Br J Haematol 2002;118:893-8.
22. Banyatsuppasin W, Butthep P, Atichartakarn V, Thakkinstian A, Archararit N, Pattanapanyasat K, Chuncharunee S. Activation of mononuclear phagocytes and its relationship to asplenia and phosphatidylserine exposing red blood cells in hemoglobin E/β-thalassemia patients. Am J Hematol 2011;86:89-92.
23. Garozzo G, Distefano R, Miceli A, Bonomo P. Detection of ICAM-1, ICAM-2, ICAM-3, PECAM-1 and VCAM-1, evaluation of hypercoagulable state and platelet aggregation in hemoglobinopathy patients with erythroblasts. Haematologica 2001;86:778-9.
24. Atichartakarn V, Chuncharunee S, Chandanamattha P, Likittanasombat K, Aryurachai K. Correction of hypercoagulability and amelioration of pulmonary arterial hypertension by chronic blood transfusion in an asplenic hemoglobin E/β-thalassemia patient. Blood 2004;103:2844-6.
25. Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned MS, El-Chafic AH, Fasulo MR, Cappellini MD. Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood 2010;115:1886-92.
26. Du ZD, Roguin N, Milgram E, Saab K, Koren A. Pulmonary hypertension in patients with thalassemia major. Am Heart J 1997;134:532-7.
27. Daniels LB, Maisel AS. Natriuretic peptides. J Am Coll Cardiol 2007;50:2357-68.
28. Yasue H, Yoshimura M, Sumida H, Kikuta K, Kugiyama K, Jougasaki M, Ogawa H, Okumura K, Mukoyama M, Nakao K. Localization and mechanism of secretion of B-type natriuretic peptide in comparison with those of A-type natriuretic peptide in normal subjects and patients with heart failure. Circulation 1994;90:195-203.
29. Nagaya N, Nishikimi T, Okano Y, et al. Plasma brain natriuretic peptide levels increase in proportion to the extent of right ventricular dysfunction in pulmonary hypertension. J Am Coll Cardiol 1998;31:202-8.
30. Atichartakarn V, Chuncharunee S, Archararit N, Udomsubpayakul U, Aryurachai K. Intravascular hemolysis, vascular endothelial cell activation, and thrombophilia in splenectomized patients with hemoglobin E/β-thalassemia disease. Acta Haematol 2014;132:100-107.
31. Westerman M, Pizzey A, Hirschman J, et al. Microvesicles in haemoglobinopathies offer insights into mechanisms of hypercoagulability, haemolysis and the effects of therapy. Br J Haematol 2008;142:126-35.
32. Karimi M, Musallam KM, Cappellini MD, Daar S, El-Beshlawy A, Belhoul K, Saned MS, Temraz S, Koussa S, Taher AT. Risk factors for pulmonary hypertension in patients with thalassemia intermedia. Eur J Intern Med 2011;22:607-10.
33. Bazzoni F, Beutler B. The tumor necrosis factor ligand and receptor families. N Engl J Med 1996;334:1717-25.
34. Nouraie M, Lee JS, Zhang Y, et al. The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe. Haematologica 2013;98:464-72.