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Pulmonary hypertension in patients with hematological disorders following splenectomy.
MedLine Citation:
PMID:  23100974     Owner:  NLM     Status:  PubMed-not-MEDLINE    
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.
Kanjaksha Ghosh; V Meera; Farah Jijina
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Publication Detail:
Type:  Journal Article     Date:  2009-07-05
Journal Detail:
Title:  Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion     Volume:  25     ISSN:  0971-4502     ISO Abbreviation:  Indian J Hematol Blood Transfus     Publication Date:  2009 Jun 
Date Detail:
Created Date:  2012-10-26     Completed Date:  2012-10-29     Revised Date:  2013-05-30    
Medline Journal Info:
Nlm Unique ID:  9425818     Medline TA:  Indian J Hematol Blood Transfus     Country:  India    
Other Details:
Languages:  eng     Pagination:  45-8     Citation Subset:  -    
National Institute of Immunohaematology(ICMR), KEM Hospital, Parel Mumbai, 400 012 India.
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