Abstract
Prevalence of pulmonary arterial hypertension (PAH) was studied by echocardiography and Doppler in 43 splenectomized patients with various disorders 1–20 years after splenectomy. Pulmonary arterial hypertension was detected only in thalassemia major, intermedia, hereditary sphereocytosis and myelofibrosis groups comprising a total of 21 patients. Six patients out of 21 was found to have PAH with mean pulmonary arterial pressure of 46.28 ± 28.17 mm of Hg. Twenty-one controls having similar duration and type of disease also were assessed for PAH in this case control study 3/21 had pulmonary arterial hypertension in this control group. The difference in number of patients showing pulmonary hypertension between case and control was not statistically significant (Chi square test P=0.29) though the difference in pulmonary arterial pressure between case and control were significantly different (t test P<0.0029) with control group showing a mean pulmonary arterial pressure of 25 ± 19 mm Hg.
Platelet count in the splenectomized group was significantly higher (P=0.0029) than the controls. Pulmonary thromboembolism was equally high in the PAH patients with and without splenectomy. Patients undergoing splenectomy due to trauma, immune thrombocytopenia, sideroblastic anemia, extrahepatic portal hypertension, autoimmune hemolytic anemia did not show PAH after splenectomy even years after the procedure PAH following splenectomy is common after certain disorders and control patients in these diseases have tendency to develop PAH even without splenectomy. Pulmonary thromboembolism may be an important pathophysiological mechanism leading to this condition. Patients having hemolytic anemia and myelofibrosis should have regular evaluation of pulmonary arterial pressure whether he/she has been splenectomized or not.
This is particularly important as availability of phosphodiesterase inhibitors such as sildenafil allows one to manage these cases.
Keywords: Pulmonary arterial hypertension (PAH), Splenectomy, Thalassemia major, Thalassemia intermedia, Myelofibrosis, Pulmonary thromboembolism
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References
- 1.Hoeper M.M., Niedermeyer J., Hoffmeyer F., Flemming P., Fabel H. Pulmonary hypertension after splenectomy? Ann Intern Med. 1999;16:793. doi: 10.7326/0003-4819-130-6-199903160-00014. [DOI] [PubMed] [Google Scholar]
- 2.Grisaru D, Rachmilewitz EA, Mosseri M, Gotsman M, Lafari JS, Okon E, Goldfarb A, Hasin Y (2004) Cunningham and Elliott Vichinsky; cardiopulmonary assessment in beta thalassemia major. ASH Education Programme Book [DOI] [PubMed]
- 3.Aessopos A., Farmakis D., Deftereos S., Tsironi M., Tassiopoulos S., Moyssakis I., Karagiorga M. Thalassemia heart disease. a comparative evaluation of thalassemia major and thalassemia intermedia. Chest. 2005;127:1523–1530. doi: 10.1378/chest.127.5.1523. [DOI] [PubMed] [Google Scholar]
- 4.Atichartakarn V., Likittanasombat K., Chuncharunee S., Chandanamattha P., Worapongpaiboon S., Angchaisuksiri P. Pulmonary arterial hypertension in previously splenectomized patients with beta thalassemia disorders. Int J Haematol. 2003;78:139–145. doi: 10.1007/BF02983382. [DOI] [PubMed] [Google Scholar]
- 5.Aessopos A., Farmakis D., Karagiorga M., Voskaridou E., Loutradi A., Hatziliami A., Joussef J., Rombos J., Loukopuolos D. Cardiac involvement in thalassemia intermedia: a multicentre study. Blood. 2001;97:3411–3416. doi: 10.1182/blood.V97.11.3411. [DOI] [PubMed] [Google Scholar]
- 6.Aessopos A., Farmakis D. Pulmonary hypertension in beta thalassemia. Ann N Y Acad Sci. 2005;1054:342–349. doi: 10.1196/annals.1345.041. [DOI] [PubMed] [Google Scholar]
- 7.Jais X., Loos V., Jardim C., Siton O., Parent F., Hamid A., Fadel E., Dartevelle P., Simmonneau G., Humbert M. Splenectomy and chronic thromboembolic pulmonary hypertension. Thorax. 2005;60:1031–1034. doi: 10.1136/thx.2004.038083. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Peacock A.J. Pulmonary hypetension after splenectomy: a consequence of loss of the splenic filter or is there something more? Thorax. 2005;60:983–984. doi: 10.1136/thx.2005.043216. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Kisanuki A., Kietthubthew S., Asada Y., Maruthsuka K., Funahara Y., Sumiyoshi A. Intravenious injectionof sonicated blood induces pulmonary microthromboembolism in rabbits with ligation of the splenic artery. Thromb Res. 1997;85:95–103. doi: 10.1016/S0049-3848(96)00226-5. [DOI] [PubMed] [Google Scholar]
- 10.Chou R., DeLoughery T.G. Recurrent thromboembolic diseae following splenectomy for pyruvate kinase deficiency. Am J Haematol. 2001;67(3):197–199. doi: 10.1002/ajh.1107. [DOI] [PubMed] [Google Scholar]
- 11.Jardine D.L., Laing A.D. Delayed pulmonary hypertension following splenectomy for congenital spherocytosis. Intern Med J. 2004;34:214–216. doi: 10.1111/j.1444-0903.2004.00580.x. [DOI] [PubMed] [Google Scholar]
- 12.Morris Cr., Kuypers F.A., Kato G.J., Lavrisha L., Larkin S., Singer T., Vichinsky E.P. Hemolysis associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2005;1054:481–485. doi: 10.1196/annals.1345.058. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 13.Bidot C.J., Jy W., Horstman L.L., Ahn E.R., Yamiz M., Ahn Y.S. Anti phospholipid antibodies (APLA) in immune thrombocytopenic purpura (ITP) and antiphospholipid syndrome (APS) Am J Haematol. 2006;81:391–96. doi: 10.1002/ajh.20571. [DOI] [PubMed] [Google Scholar]
- 14.Eldor, Rachmilewitz E.A. The hypercoagulable state in thalassemia. Blood. 2002;99:36–43. doi: 10.1182/blood.V99.1.36. [DOI] [PubMed] [Google Scholar]
- 15.Sonakul D., Fucharoen S. Pulmonary thromboembolism in thalassemic patients. South East Asian J Trop Med Public Health. 1992;23:25–28. [PubMed] [Google Scholar]
- 16.Dingli D., Utz J.P., Krowka M.J., Oberg A.L., Tefferi A. Unexplained pulmonary hypertension in chronic myeloproliferative disorders. Chest. 2001;120:801–808. doi: 10.1378/chest.120.3.801. [DOI] [PubMed] [Google Scholar]
- 17.Rubin L.J. Primary pulmonary hypertension. New Engl J Med. 1997;336:111–117. doi: 10.1056/NEJM199701093360207. [DOI] [PubMed] [Google Scholar]
- 18.Michelakis E.D., Tymchak W., Noga M., Webster L., Wu X.C., Lien D., et al. Long term treatment with oral sildenafil is safe and improves functional capacity and haemodynamics in patients with primary pulmonary hypertension. Circulation. 2003;108:2066–2069. doi: 10.1161/01.CIR.0000099502.17776.C2. [DOI] [PubMed] [Google Scholar]