Idiopathic Autoimmune Hemolytic Anemia a Report of Two Cases in Infants

特發性自體免疫溶血性貧血於嬰兒之兩例報告

林季珍(Ghi-Jen Lin) ； 林國信(Kuo-Sin Lin) ； 李廷堅(Ting-Chien Lee)

《Acta Paediatrica Sinica》 19卷1期 (1978/03) Pp. 45-54

摘要

於孩童，自體免疫溶血性貧血係屬較少見的溶血性貧血。孩童期發生的自體免疫溶血性貧血常繼發於某種細菌或病毒感染，只有小部分屬於特發性。因影響此病溶血的因素很多，故其臨床經過多變，且其對治療之反應也不易判定。本病大都先採用類固醇治療，無效時才考慮脾臓摘除，胸腺摘除或免疫抑制等療法。
本文報告兩例嬰兒特發性自體免疫溶血性貧血的病例。此兩例在發病時均有重度貧血及輕度黃疸，在發病時均未有細菌或病毒感染之徵象亦未曾服用藥物。兩例均由陽性庫姆斯試驗(Coomb' test)得到證實。第1例爲7個月女嬰，發病時直接及間接庫姆斯試驗均爲陽性，臨床上對類固醇之治療反應不佳，但給予cyclophosphamide後，血液相有明顯改善，却不幸死於肺炎，在整個臨床經過中，直接庫姆斯試驗未轉爲陰性。第2例爲8個月男嬰，發病時直接庫姆斯試驗爲陽性，間接庫姆斯試驗則爲陰性。臨床上對類固醇反應甚佳，服藥1個月後，血液相已恢復正常。本例又繼續服用小量類固醇3個月直至庫姆斯試驗轉爲陰性爲止。追踪至距發病之時已有18個月，尚無再發。
文中有關本病與細菌或病毒感染的關係，各種療法之使用及其效果，以及庫姆斯試驗於臨床上之實用價值，亦有討論。

並列摘要

Two cases of idiopathic autoimmune hemolytic anemia, proved by the positive Coombs' test, are presented. Both cases showed extreme anemia and mild jaundice without preceding bacterial or viral infections. No history of drug ingestion before the onset could be elicited in both cases. The first case, a 7-month-old female baby, having positive direct and indirect Coombs' tests at the onset, was unresponsive to prednisolone therapy, but a prompt and progressive increase of the hemoglobin value was observed after the institution of cyclophosphamide. The direct Coombs' test never reverted to negative in the course of the disease, and the patient died of pneumonia 3 months after the onset of the illness. The second case was an 8-month-old male baby with only a positive direct Coombs' test, but a negative indirect Coombs' test initially. The blood counts returned to normal about 1 month after prednisolon therapy. A low dose of prednisolone was maintained for another 3 months until the Coombs' test gradually reverted to negative. A follow-up study for 18 months has thus far revealed no recurrence.

並列關鍵字

immunohemolytic anemia ； idiopathic ； infantile