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The Response to Erythropoietin in Hemodialysis Patients with Thalassemia Diagnosed by Genomic DNA Analysis
尿毒症患者併地中海型貧血對紅血球生成素反應之研究
摘要
尿毒症患者併地中海貧血，對紅血球生成素(erythropoietin)需要量的多寡仍有爭議。雖然，一些研究報告偏向於這類病人需要較高的劑量，才能達到理想的血容積(Hct)。但是，這些文章中病人的樣本數太少，且對地中海型貧血之診斷，主要依據血紅素電泳(Hb electrophoresis)及紅血球形態的方法，其準確性不若基因分析法(genomic DNA analysis )。此篇研究，則是藉由分析台灣地區常見的地中海型貧血之基因型，找出本院血液透析病患合併地中海型貧血者，探究他們對紅血球生成素反與之差異。而我們選取30% 為目標血容積(target hematocrit)，這個標準符合目前健保政策及美國食品和藥物管理局(FDA)所認可之基本需求。 本實驗找出152位長期接受血液透析至少個月的病人，他們的血容積持續三個月小於30%，同時排除一些會影響紅血球生成素產生或反應的因素(地中海型貧血除外)。這此病人若平 均血球體積(MCV)小於80 f1，則接受地中海貧血之基因分析。結果我們發現17位地中海型貧血患者(13位為甲型，4位為乙型)，而非地中海型貧血組則有135位病人。這兩組病人，皆以30%為目標血容積，根據其血容積的高低，每週接受不同劑量之紅血球生成素皮下注射。然而，在六個月後我們發現，此兩組病人在最終的血容積(28.4%±2.4% 對 29.2±2.8%)和紅血球生成素的用量上(41.2±14.5 對 38.6±13.6, x 2000u)，並無統計學之差異。因此，就實際臨床施行觀點而言，以目標血容積為30%的前提下，血液透析病患有無地中海分血，他們對紅血球成素的需求量，在統計上是相同的。從這結果我們推測，若血液透析病人併地中海貧血有難以矯正之嚴重貧血，可能其屬於重度地中海貧血或者有其他的因素，造成病人對紅血球生成素反應不佳。
並列摘要
The availability of recombinant human erythropoietin (rHuEpo)has effectively alleviated uremic anemia. In thalassemia patients on chronic dialysis, the response to rHuEpo still is in debate although a higher dose of rHuEpo requirement was announced in several small-scale reports. The purpose of our study was to assess the response to rHuEpo in hemodialysis patients with thalassemia to achieve the target hematocrit of 30% guided by the practical need and policy of health insurance. Patients (152) with end stage renal disease (ESRD) on regular hemodialysis at our institution for more than 3 months and whose Hct were les than target level of 30% fro 3 months were included in our study. Factors that may influence the endogenous production of Epo or response to rHuEpo therapy were excluded. We identified 17 thalas-semia patients (13 α-thalassemia, 4 ß-thalassemia) and 135 non-thalasemia patients. There no statistical differences between the two groups in the age, duration of dialysis, the level of ferritin and the Hct level at the beginning of the study. However, after aministration of rHuEpo for 6 months, the Hct at the end of study (28.4%±2.4% versus 29.2±2.8%) and the total dosage of rHuEpo use (41.2±14.5 versus 38.6±13.6, x2000u; equal to 3204.4±1127.7 vs. 3002.2±1057.7, U/week) between the two groups exhibited no significant difference. In conclusion, from the economic point of view and practical purpose, to achieve the target hematocrit of 30%, the requirement and response to rHuEpo in thalassemia minor and non-thalassemia patients with ESRD on chronic hemodialysis have no significant difference in our study.