Atichartakarn Vichai, Chuncharunee Suporn, Chandanamattha Pakorn, Likittanasombat Khanchit, Aryurachai Katcharin
Division of Hematology, Department of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Blood. 2004 Apr 1;103(7):2844-6. doi: 10.1182/blood-2003-09-3094. Epub 2003 Nov 26.
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/beta-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 microg/L (normal, 3 +/- 2.4 microg/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.
除了使用华法林、乙酰水杨酸、去铁胺进行其他持续治疗以及采取其他支持措施外，还给一位接受脾切除的血红蛋白E/β地中海贫血且患有肺动脉高压（PHT）的女性长期输注浓缩红细胞。采用连续测量血浆凝血酶 - 抗凝血酶III复合物（TAT）水平及右侧心导管检查来监测治疗后的变化。血浆TAT水平从7.5降至3.8μg/L（正常范围为3±2.4μg/L），肺血管阻力（PVR）从553.8降至238.6达因·秒·厘米⁻⁵（正常范围为67±30达因·秒·厘米⁻⁵），平均肺动脉压从51降至32mmHg（正常范围为9至19mmHg），这些变化同步发生。长期输血使血浆TAT水平所反映的血液高凝状态恢复正常，这可能是PVR升高得到改善的基础，PVR升高继发于血栓形成性肺动脉病，进而导致PHT。
