Pulmonary hypertension risk in patients with hemoglobin H disease: Low Incidence and absence of correlation with splenectomy

Acta Haematologica

Volumn 130, Issue 3, 2013, Pages 153-159

  Yin, Xiao Lin ^a   Zhang, Xin Hua ^a   Wu, Zhi Kui ^b   Zhao, Dong Hai ^a   Zhou, Ya Li ^a   Yu, Yue Hua ^a   Liu, Tie Niu ^a   Fang, Su Ping ^b   Zhou, Tian Hong ^a   Wang, Li ^a   Huang, Jie ^a  

^a The 303rd Hospital of PLA   (China)

^b INSTITUTE OF CHINESE MATERIA MEDICA   (China)

Author keywords

Alpha thalassemia; Pulmonary hypertension; Splenectomy

Indexed keywords

ADOLESCENT; ADULT; AGED; ARTICLE; BETA THALASSEMIA; CARDIOVASCULAR PARAMETERS; CHILD; CONTROLLED STUDY; DISEASE ASSOCIATION; ERYTHROCYTE COUNT; FEMALE; HEMOGLOBIN H DISEASE; HUMAN; INCIDENCE; MAJOR CLINICAL STUDY; MALE; PRESCHOOL CHILD; PREVALENCE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; RISK ASSESSMENT; SCHOOL CHILD; SPLENECTOMY; THALASSEMIA INTERMEDIA; THROMBOCYTE COUNT; AGE; REGRESSION ANALYSIS; RISK FACTOR;

ADOLESCENT; ADULT; ALPHA-THALASSEMIA; CASE-CONTROL STUDIES; CHILD; FEMALE; HUMANS; HYPERTENSION, PULMONARY; MALE; MIDDLE AGED; RISK FACTORS; SPLENECTOMY;

EID: 84878097741     PISSN: 00015792     EISSN: 14219662     Source Type: Journal    
DOI: 10.1159/000347177     Document Type: Article

References (29)

1. Weatherall DJ: Keynote address: the challenge of thalassemia for the developing countries. Ann NY Acad Sci 2005; 1054: 11-17.
2. 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: 1886-1892.
3. Barnett CF, Hsue PY, Machado RF: Pulmonary hypertension: an increasingly recognized complication of hereditary hemolytic anemias and HIV infection. JAMA 2008; 299: 324-331.
4. Farmakis D, Aessopos A: Pulmonary hypertension associated with hemoglobinopathies: prevalent but overlooked. Circulation 2011; 123: 1227-1232.
5. Singer ST, Kuypers FA, Styles L, Vichinsky EP, Foote D, Rosenfeld H: Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state. Am J Hematol 2006; 81: 670-675.
6. Taher AT, Musallam KM, Karimi M, El-Beshlawy A, Belhoul K, Daar S, Saned M, Cesaretti C, Cappellini MD: Splenectomy and thrombosis: the case of thalassemia intermedia. J Thromb Haemost 2010; 8: 2152-2158.
7. Musallam KM, Cappellini MD, Wood JC, Motta I, Graziadei G, Tamim H, Taher AT: Elevated liver iron concentration is a marker of increased morbidity in patients with beta thalassemia intermedia. Haematologica 2011; 96: 1605-1612.
8. Morris CR, Gladwin MT, Kato GJ: Nitric oxide and arginine dysregulation: a novel pathway to pulmonary hypertension in hemolytic disorders. Curr Mol Med 2008; 8: 620-632.
9. Gladwin MT, Sachdev V, Jison ML, Shizukuda Y, Plehn JF, Minter K, Brown B, Coles WA, Nichols JS, Ernst I, Hunter LA, Blackwelder WC, Schechter AN, Rodgers GP, Castro O, Ognibene FP: Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med 2004; 350: 886-895.
10. Tan JA, Lee PC, Wee YC, Tan KL, Mahali NF, George E, Chua KH: High prevalence of alpha-and beta-thalassemia in the Kadazandusuns in East Malaysia: challenges in providing effective health care for an indigenous group. J Biomed Biotechnol 2010, DOI: 10. 1155/ 2010/706872.
11. Xiong F, Sun M, Zhang X, Cai R, Zhou Y, Lou J, Zeng L, Sun Q, Xiao Q, Shang X, Wei X, Zhang T, Chen P, Xu X: Molecular epidemiological survey of haemoglobinopathies in the Guangxi Zhuang Autonomous Region of southern China. Clin Genet 2010; 8: 139-148.
12. Xu XM, Zhou YQ, Luo GX, Liao C, Zhou M, Chen PY, Lu JP, Jia SQ, Xiao GF, Shen X, Li J, Chen HP, Xia YY, Wen YX, Mo QH, Li WD, Li YY, Zhuo LW, Wang ZQ, Chen YJ, Qin CH, Zhong M: The prevalence and spectrum of alpha and beta thalassaemia in Guangdong Province: implications for the future health burden and population screening. J Clin Pathol 2004; 57: 517-522.
13. Chui DH, Fucharoen S, Chan V: Hemoglobin H disease: not necessarily a benign disorder. Blood 2003; 101: 791-800.
14. Yin XL, Zhang XH, Zhou TH, Zhang TL, Luo RG, Wang L, Zhou YL, Chen YS, Kong XJ, Liang B, He YY, Peng L, Lu LB, Fang SP, Wu ZK: Hemoglobin H disease in Guangxi province, southern China: clinical review of 357 patients. Acta Haematol 2010; 124: 86-91.
15. Yin XL, Wu ZK, He YY, Zhou TH, Zhou YL, Zhang XH: Treatment and complications of thalassemia major in Guangxi, southern China. Pediatr Blood Cancer 2011; 57: 1174-1178.
16. Yang XY, Qu Q, Yang TY, Chan WC, Chu JX, Chen Z, Ning AL, Liu FJ, Lin ZX, Zhou YL: Treatment of the thalassemia syndrome with splenectomy. Hemoglobin 1988; 12: 601-608.
17. Phrommintikul A, Sukonthasarn A, Kanjanavanit R, Nawarawong W: Splenectomy: a strong risk factor for pulmonary hypertension in patients with thalassaemia. Heart 2006; 92: 1467-1472.
18. Morris CR, Kim HY, Trachtenberg F, Wood J, Quinn CT, Sweeters N, Kwiatkowski JL, Thompson AA, Giardina PJ, Boudreaux J, Olivieri NF, Porter JB, Neufeld EJ, Vichinsky EP: 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: 3794-3802.
19. Camaschella C, Cappellini MD: Thalassemia intermedia. Haematologica 1995; 80: 58-68.
20. Rigas DA, Koler RD: Decreased erythrocyte survival in hemoglobin H disease as a result of the abnormal properties of hemoglobin H: the benefit of splenectomy. Blood 1961; 18: 1-17.
21. Hathirat P, Isarangkura P, Numhom S, Opasathien P, Chuansumrit A: Results of the splenectomy in children with thalassemia. J Med Assoc Thai 1989; 72(suppl 1):133-138.
22. Amoozgar H, Farhani N, Karimi M: Early echocardiographic findings in beta-thalassemia intermedia patients using standard and tissue Doppler methods. Pediatr Cardiol 2011; 32: 154-159.
23. 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 beta thalassemia intermedia. Eur J Intern Med 2011; 22: 607-610.
24. Lithanatudom P, Leecharoenkiat A, Wannatung T, Svasti S, Fucharoen S, Smith DR: A mechanism of ineffective erythropoiesis in beta-thalassemia/Hb E disease. Haematologica 2010; 95: 716-723.
25. Danise P, Amendola G, Di CR, Cillari E, Gioia M, Di PA, Avino D, Rigano P, Maggio A: Nucleated red blood cells and soluble transferrin receptor in thalassemia syndromes: relationship with global and ineffective erythropoiesis. Clin Chem Lab Med 2009; 47: 1539-1542.
26. Aessopos A, Kati M, Farmakis D: Heart disease in thalassemia intermedia: a review of the underlying pathophysiology. Haematologica 2007; 92: 658-665.
27. Amoozgar H, Farhani N, Karimi M: Risk factors for pulmonary hypertension in patients with thalassemia intermedia. Eur J Haematol 2010; 85: 549-551.
28. Winichagoon P, Fucharoen S, Wasi P: Increased circulating platelet aggregates in thalassaemia. Southeast Asian J Trop Med Public Health 1981; 12: 556-560.
29. Sonakul D, Pacharee P, Laohapand T, Fucharoen S, Wasi P: Pulmonary artery obstruction in thalassaemia. Southeast Asian J Trop Med Public Health 1980; 11: 516-523.