scholarly journals Benefits of chronic blood transfusion in hemoglobin E/β thalassemia with pulmonary arterial hypertension

2014 ◽  
pp. 411 ◽  
Author(s):  
Nonlawan Chueamuangphan ◽  
Jayanton Patumanond ◽  
Wattana Wongtheptien ◽  
Weerasak Nawarawong ◽  
Apichard Sukornthasarn ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Blood Transfusion ◽  
Arterial Hypertension ◽  
Beta Thalassemia ◽  
Hemoglobin E ◽  
Pulmonary Arterial

Correction of hypercoagulability and amelioration of pulmonary arterial hypertension by chronic blood transfusion in an asplenic hemoglobin E/β-thalassemia patient

Blood ◽  
2004 ◽  
Vol 103 (7) ◽  
pp. 2844-2846 ◽  
Author(s):  
Vichai Atichartakarn ◽  
Suporn Chuncharunee ◽  
Pakorn Chandanamattha ◽  
Khanchit Likittanasombat ◽  
Katcharin Aryurachai
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Blood Transfusion ◽  
Arterial Hypertension ◽  
Thalassemia Patient ◽  
Hemoglobin E ◽  
Packed Red Blood Cells ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Thrombin Antithrombin Iii Complex ◽  
Serial Measurements

Abstract Chronic transfusion of packed red blood cells, in addition to other ongoing treatment with warfarin, acetyl salicylic acid, desferrioxamine, and other supportive measures, was given to a splenectomized hemoglobin E/β-thalassemia woman with pulmonary arterial hypertension (PHT). Serial measurements of plasma thrombin-antithrombin III complex (TAT) levels and right-sided cardiac catheterization were used to monitor changes after treatment. Reduction of plasma TAT levels from 7.5 to 3.8 μg/L (normal, 3 ± 2.4 μg/L), pulmonary vascular resistance (PVR) from 553.8 to 238.6 dyne.sec.cm-5 (normal, 67 ± 30 dyne.sec.cm-5), and mean pulmonary arterial pressure from 51 to 32 mm Hg (normal, 9 to 19 mm Hg) occurred in tandem. Normalization of blood hypercoagulability as reflected in plasma TAT level by chronic blood transfusion was the likely basis for improvement of increased PVR, being secondary to thrombotic pulmonary arteriopathy and subsequently PHT. (Blood. 2004;103: 2844-2846)

Prevalence and Predictors of Pulmonary Arterial Hypertension in Hemoglobin E/ β-Thalassemia Patients.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2015-2015 ◽  
Author(s):  
Suporn Chuncharunee ◽  
Vichai Atichartakarn ◽  
Napaporn Archararit ◽  
Umaporn Udomsubpayakul ◽  
Atiporn Ingsathit ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Univariate Analysis ◽  
Cellular Adhesion ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Low Grade ◽  
Ventricular Ejection Fraction ◽  
Hemoglobin E ◽  
Pulmonary Arterial

Abstract Abstract 2015 Poster Board I-1037 Introduction: Pulmonary arterial hypertension (PAH) associated with thalassemia (Thal) hemoglobinopathy is now an accepted clinical entity. Most of the reports are in sickle cell disease and splenectomized β-Thal patients. Once manifested, it connotes poor prognosis. We herein present its prevalence and predictors in hemoglobin E/β-Thal (E/β-Thal) patients. Patients and Methods: One hundred and ten clinically stable E/β-Thal outpatients, on no medication aside from folic acid and who received no blood transfusion in the preceding 4 weeks were studied. All gave written informed consent, and study protocol was approved by the institution ethics committee on studies in humans (#0774/2548). Echocardiogram was used to estimate systolic PA pressure (SPAP). PAH was defined as an estimated SPAP ≥36 mmHg. Clinical features and laboratory data were stratified according to the presence or absence of PAH, and statistical analysis was done by STATA version 10 (Stata Corp, Texas), considering a P value <0.05 as statistically significant. Predictors of PAH were considered in univariate analysis. Results: There were 110 patients, 56 of whom were female and 61 were asplenic. PAH was present in 41 patients (37.3%), all of whom had normal left ventricular ejection fraction. There was no gender difference between the 2 groups (p=0.055). Selected statistically significant results are shown in the table. Conclusions: Prevalence of PAH in E/β-Thal patients is 37.3% without gender preponderance. Predictors are asplenia, more severe hemolysis, higher number of circulating (activated) platelets and nucleated RBCs, increased chronic low grade inflammation and increased cellular adhesion between blood and endothelial cells. These changes could facilitate development of thrombotic pulmonary arteriopathy, the underlying basis of PAH. Serum NT pro BNP assay can be utilized as a predictor or a screener of PAH in these patients. Disclosures: No relevant conflicts of interest to declare.

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