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小儿重症再生障碍性贫血(下称重症再障)预后不良,在日本约有60％患者死于发病初期,长期生存者约占30％。因而,虽把骨髓移植作为有组织相容同胞的重症再障患儿的首选疗法,但实际上往往没有组织相容的供髓者。 已知有在骨髓移植的同时进行免疫抑制后恢复自身造血功能的病例,也有在免疫抑制后才开始生效的双胞胎间的骨髓移植的病例。还有再障患者T细胞在体外抑制造血干细胞形成集落的报导。因此,免疫抑制可能参与部分再障病情的发病机制,期望免疫抑制疗法能用于重症再障的治疗。特别是抗淋巴细胞球蛋白(ALG)疗法,其疗效仅次于骨髓移植,故不能进行骨髓移植的病例,应首选此疗法。
Children with severe aplastic anemia (hereinafter referred to as severe aplastic anemia) poor prognosis in Japan, about 60% of patients died of early onset, long-term survival of about 30%. Thus, although bone marrow transplantation is the preferred therapy for pediatric patients with histopathologically compatible aplastic anemia, in practice, there is often no tissue compatible donor. There are known cases in which self-hematopoietic function is restored after immunosuppression is performed in addition to bone marrow transplantation, and there are also cases of bone marrow transplantation between twins that started taking effect after immunosuppression. There are reports of patients with aplastic anemia in which T cells inhibit the formation of colonies of hematopoietic stem cells in vitro. Therefore, immunosuppression may be involved in the pathogenesis of partial aplastic anemia and immunosuppressive therapy is expected to be useful in the treatment of severe aplastic anemia. In particular, anti-lymphocyte globulin (ALG) therapy, its efficacy is second only to bone marrow transplantation, it can not be bone marrow transplantation cases, this therapy should be the first choice.