Choulot J J, Parent Y, Etcharry F, Saint-Martin J, Mensire A
Service de pédiatrie, centre hospitalier général, Pau, France.
Arch Pediatr. 1996 Aug;3(8):789-91. doi: 10.1016/0929-693x(96)82162-2.
The previously reported cases of giant cell hepatitis with autoimmune hemolytic anemia were improved by prednisone plus azathioprine.
A 14-month-old boy suffered from giant cell hepatitis with auto-immune hemolytic anemia and positive direct Coombs test. Prednisone and azathioprine administration improved the liver disease but failed to control hemolysis so that repeated blood transfusions were necessary. Persistance of severe degree of hemolysis required splenectomy that was promptly and definitively effective. Azathioprine and prednisone were pursued for a total duration of five years. Twelve years after the onset of the disease, the child is well without any treatment.
This is the first reported case of such an association in which poorly controlled auto-immune hemolytic anemia benefited from splenectomy.
先前报道的巨细胞性肝炎合并自身免疫性溶血性贫血的病例通过泼尼松加硫唑嘌呤治疗后病情得到改善。
一名14个月大的男孩患有巨细胞性肝炎合并自身免疫性溶血性贫血，直接抗人球蛋白试验呈阳性。给予泼尼松和硫唑嘌呤治疗后肝病有所改善，但未能控制溶血，因此需要反复输血。由于严重溶血持续存在，需要进行脾切除术，该手术迅速且疗效确切。硫唑嘌呤和泼尼松总共使用了五年。疾病发作十二年后，该患儿未经任何治疗，情况良好。
这是首次报道的此类关联病例，其中控制不佳的自身免疫性溶血性贫血通过脾切除术获益。
