小兒再生不良性貧血症之臨床觀察

Clinical Observation of Hypoplastic Anemia in Childhood

陳添享(T. S. Chen) ； 黃文雄(W. H. Huang) ； 呂志忠(C. C. Lu)

《Acta Paediatrica Sinica》 11卷4期 (1970/12) Pp. 152-162

摘要

著者等就自民國四十八年至民國五十八年共計十一年間住進高雄醫學院小兒科，被診斷爲再生不良性貧血症58例作臨床觀察分析結果如下：
1．男性多於女性，男女之比例3.83:1, 年齡最小爲9個月男嬰，其中11~14歲之患兒者33例(56.89%)居首位，在年度上沒有增加。
2．入院時之主要臨床症狀多爲緩慢出現，隨疾病之進行而加割，一般症狀有臉色蒼白、機能性心雜音、皮膚出血、齒根出血、鼻出血、發熟、口腔粘膜出血、疲倦、腹痛等症狀。
3．入院時之末梢血液檢查，紅血球、白血球、血小板三要素皆顯著低下，其範圍分別爲：紅血球40×10^4~325×10^4/立方公厘，平均141.4×10^4/立方公厘，血紅素2-10gm/dl，平均4.61gm/dl；白血球1,100~9,000/立方公厘，平均3,970/立方公厘；血小板6,000~180,000/立方公厘，平均55,391.9/立方公厘；綱織紅血球0~11‰之間，大部份在3‰以下；出血時間延長，2'15''~80'30''，平均21'30''。
4．骨髓像外觀上呈現脂肪性骨髓，細胞數爲低細胞羣(Hypocellular)或無細胞羣(Acellula)，有核細胞總數明顯降低，範圍爲1,800~76,700/立方公厘，平均17,710/立方公厘，而早期造血細胞欠缺或很少，主要細胞爲淋巴球或少數分葉白血球。
5．治療方面以輸血及腎上腺荷爾蒙併用較好，再加上男性荷爾蒙三者併用，預後更佳，生存期也較長。
6．通信方式追蹤獲知25例狀況，其中死亡者17例(68%)，他們皆在一年內死亡，而健存者8例。

並列摘要

During the past 11 years, 58 cases of hypoplastic anemia in infancy and childhood were admitted to the Kaohsiung Medical College Hospital. The clinical observations are summarized as follows:
1. There was a sex predominance of male with a ratio of 3.83:1. The youngest patient was 9 months old, however, more than half of the patients were from 11 to 14 years of age. No tendency of increase in incidence with the progress of years was noted.
2. The clinical symptoms and signs on admission included bland pallor of insidious onset, functional heart murmur, echymosis, petechia, gum bleeding, epistaxis, low grade fever, mucosal bleeding or ulcer, dizziness, malaise etc., The above manifestations became worse with the progress of the disease.
3. The peripheral blood picture on admission showed pancytopenia with their range varying as follows: RBC 40×40^4-325×10^4/mm^3 (Av. 141.41×10^4/mm^3), Hb 2-10gm/dl (Av. 4.61gm/dl), WBC 1,100-9,000/mm^3 (Av. 3,974/mm^3), platelets 6,000-180,000/mm^3 (Av. 59,997/mm^3), reticulocyte 0-11‰ (most cases below 3‰), bleeding time prolonged 2'15''-80'30'' (Av. 21'30''). The coagulation time, RBC fragility test, liver function test were almost normal. The serum iron value increased ranging from 60γ/dl-320γ/dl (Av. 217.12γ/dl) in 26 examined cases. In about 72.11% of these cases, the serum iron values were above 180γ/dl.
4. The appearance of bone marrow was fatty, whitish in character and the microscopic finding was hypo cellular with a decrease or lack of stem cells. But the lymphocytes increased in most cases. The total nucleated cell count showed a marked decrease ranging from 1,800/mm^3 to 76,700/mm^3 (Av. 17,710/mm^3).
5. In treatment, it seemed better to combine blood transfusion and steroid hormone. Additihn testosterone seemed to be more effective in terms of response, prognosis and survival rate.
6. Among 25 cases followed up by letters, 17 (64%) of them died within one year, while 8 cases survived the observation period of 1 to 10 years.