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[成人重型再生障碍性贫血的免疫调节治疗]

[Immunomodulating treatment of severe aplastic anemia in adults].

作者信息

Tangen J M, Andersen O K

机构信息

Hematologisk avdeling, Medisinsk klinikk, Ullevål sykehus, Oslo.

出版信息

Tidsskr Nor Laegeforen. 1993 Apr 30;113(11):1351-4.

PMID:8337623
Abstract

Immune modulating therapy has greatly improved the prognosis in aplastic anaemia, but the response to treatment varies considerably. We describe eight patients who were treated for severe aplastic anaemia. Antithymocyte globulin was given as first line treatment, followed by cyklosporine A if the patient failed to respond. Seven patients entered complete remission, but two of these relapsed later. Both developed paroxysmal nocturnal haemoglobinuria. One patient failed to respond to therapy. She died of bone marrow failure and haemochromatosis 12 months after start of therapy. Immune modulating therapy is discussed in relation to bone marrow transplantation, which is the alternative therapy for younger patients.

摘要

免疫调节疗法极大地改善了再生障碍性贫血的预后,但治疗反应差异很大。我们描述了8例接受严重再生障碍性贫血治疗的患者。抗胸腺细胞球蛋白作为一线治疗药物,若患者无反应则随后给予环孢素A。7例患者进入完全缓解期,但其中2例后来复发。两人均发展为阵发性夜间血红蛋白尿。1例患者对治疗无反应。她在治疗开始12个月后死于骨髓衰竭和血色素沉着症。本文结合骨髓移植讨论了免疫调节疗法,骨髓移植是年轻患者的替代疗法。

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