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題名 | 骨髓增生不良症候群=Myelodysplastic Syndrome |
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作者姓名(中文) | 賴美雲; 鄭吉元; 陳琦華; | 書刊名 | 藥學雜誌 |
卷期 | 31:2=123 2015.06[民104.06] |
頁次 | 頁82-87 |
分類號 | 415.6 |
關鍵詞 | 骨髓增生不良症候群; Myelodysplastic syndrome; MDS; Azacitidine; Decitabine; Lenalidomide; |
語文 | 中文(Chinese) |
中文摘要 | 骨髓增生不良症候群 (myelodysplastic syndrome, 簡稱 MDS) 是最常見的罕見血液 疾病,好發於老年人 (≧ 65歲),男性居多。此病常併有 DNA 的不正常甲基化,導致 骨髓產生無功能的血液細胞。MDS 早期的臨床症狀是貧血,接著血小板及白血球也 會減少。臨床上 MDS 預後大多採用 IPSS 分數來評估。MDS 根據病情和病人狀況, 可給予輸血、施打 azacitidine (或 decitabine) 或是進行異體幹細胞移植的處置。對於具 有單純5q 缺乏 (deletion) 的染色體異常者,可選擇 lenalidomide。大部分 MDS 病人藉 由支持性療法 (例如輸注紅血球、血小板) 就可存活十年以上。長期輸血要注意鐵質 沉積問題,必要時應給予排鐵劑。MDS 的藥物治療中,在台灣具有衛福部核准適應 症和健保給付的只有 azacitidine。 |
英文摘要 | Myelodysplastic syndrome (MDS) is one of the most common rare hematological disorders. The majority of MDS patients are men over 65 years old. This disease is often accompanied by abnormal gene methylation, resulting in ineffective blood cells production by bone marrow. Early symptom of MDS is anemia, followed by thrombocytopenia and neutropenia. IPSS score is a generally accepted tool to evaluate the prognosis of MDS. Management of MDS includes blood transfusion, medication (azacitidine, decitabine, lenalidomide) and allogenic stem cell transplantation according to the disease status and the patient's condition. Lenalidomide is the drug of choice for patients with 5q deletion chromosome. Azacitidine is the only drug approved by TFDA and reimbursed by the NHI for the treatment of MDS in Taiwan. Most MDS patients can survive for more than 10 years only through supportive care by transfusion of red blood cells and/or platelets. Iron overload should be noted in long-term transfusion patients, and administered iron chelating drugs if necessary. |
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