小兒再生不良性貧血之臨床研究及預後之評估

The Clinical Features and Evaluation of Prognostic factors in Aplastic Anemia of Childhood

尤憲明(Shang-Ming Yue) ； 張泰琮(Tai-Tsung Chang) ； 陳添享(Tien-Siang Chen)

《Acta Paediatrica Sinica》 24卷2期 (1983/06) Pp. 106-115

摘要

自民國59年1月至民國71年12月共12年間，高雄醫學院小兒科共有再生不良性貧血病人62例。男女之比爲2.44：1。年齡之分佈大部份(77.4%)介於7-15歲之間。先天性的有3例，一爲 Fanconi氏貧血，另二例爲Diamond-Blacdfan症候羣是同胞兄弟。後天性有59例，其中79.7%致病原因不明。在25治療例中，有18例以Prednisolone 和 Testosterone治療，完全緩解率爲29.4%，部份緩解率亦爲29.4%。另7例以Prednisolone和Oxymetholone治療，完全緩解率爲25.0%，部份緩解率爲50.0%。兩種治療方式，在完全緩解率方面無顯著差異。若扣除失蹤的2例，沒有治療病例有32例，其中3例自動完全緩解佔9.4%。在25例治療例中，各血球系之緩解，以紅血球系最多，完全緩解率爲52.0%，平均達緩解期間爲13.5個月。白血球次之，完全緩解率爲47.4%，平均達緩解期間爲15.4個月。血小板最不易緩解，完全緩解率爲32.0%，平均達緩解期間爲31.3個月。在25治療例，一年存活率爲83.9%，五年存活率爲74.6%，生存期間個於3-134個月，平均50.4個月。在32無治療例中，一年存活率爲29.8%，五年存活率爲13.1%，生存期間在2天到146個月之間，平均30.8個月。可見在治療例有較高的存活率及較長的生存期間。死因主要爲敗血症(31.6%)，全身性出血(34.2 %)和顱內出血(34.2%)。在預後評估上，對初診斷時各檢查值，將已知預後的60例，分成生存例與死亡例二組，以Mann-Whitney U test 比較，得知以顆粒球絕對數或白血球數較有價值，其值愈低預後愈差。

並列摘要

Within the past 12 years, from Jan, 1970 through Dec. 1982, there were 62 cases of aplastic anemia diagnosed at the Department of pediatrics of Chung-Ho Memorial Hospital of Kaohsiung Medical College. The sex ratio of the cases was M:F=2.44:1, and the main age distribution was between 7 and 15 years old (77.4%). Among 62 cases, three of them were congenital in origin, including one case of Fanconi's anemia and two siblings of Diamond-Blackfan Syndrome. Fifty-nine cases were acquired type and 79.7% of them were etiologically unknown. Twenty-five cases had recieved medical treatment; 18 cases were treated with prednisolone and testosterone with a complete remission rate of 29.4% and a partial remission rate of 29.4%, and the other seven cases were treated with prednisolone and oxymetholone with a complete remission rate of 25.0% and partial remission rate of 50%. There was no significant difference between the two regimens in term of complete remission rate. Among 32 cases who had recieved no medical treatment, three had spontaneous complete remission (9.4%). In the 25 treated cases, there were significantly higher survival rate and longer life expectancy, 1 year survival rate was 83.9% and 5 years survival rate was 74.6%. The lift spans ranged from 3-134 months with an average of 50.4 months. In 32 untreated cases, 1 year survival rate was 29.8% and 5 years survival rate was 13.1%. Their life spans ranged from 2 days to 146 months with an average of 30.8 months. The main causes of death were sepsis (31.6%), generalized bleeding (34.2%), and intracranial bleeding (34.2%). For evaluation of prognosis, absolute granulocyte or white blood cell count is useful.

並列關鍵字

aplastic anemia ； Fanconi's anemia ； Diamond-Blackfan syndrome ； prognostic factors