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对一匹患有原发性严重联合免疫缺陷的马驹进行的重建尝试。

Attempted reconstitution of a foal with primary severe combined immunodeficiency.

作者信息

Campbell T M, Studdert M J, Ellis W M, Paton C M

出版信息

Equine Vet J. 1983 Jul;15(3):233-7. doi: 10.1111/j.2042-3306.1983.tb01776.x.

Abstract

A foal with primary severe combined immunodeficiency, diagnosed within the first two weeks of life, was maintained with its dam in semi-isolation. The foal received continuous prophylactic antibiotic therapy, plasma from a sibling hyperimmunised with equine adenovirus vaccine, and intensive general nursing care. A full sibling female was selected as a bone marrow donor on the basis of red blood cell cross-matching and mixed lymphocyte reactions. Cyclophosphamide was given before two bone marrow transfusions at 35 and 73 days of age. To prevent graft versus host disease graft versus host disease the foal was maintained on methotrexate therapy. Reconstitution was not achieved nor were there signs of graft versus host disease. The foal died suddenly four days after the second bone marrow transfer when 77 days old. It had remained clinically free of any life threatening infectious disease and at necropsy a remarkable degree of freedom from infectious disease was confirmed. The most notable necropsy findings were bilateral nephrosis and myocardial degeneration and fibrosis. The likely cause of death was an electrolyte imbalance, particularly hypokalaemia, which secondarily affected the myocardium. Renal toxicity caused by the cytotoxic drugs, especially cyclophosphamide, may have contributed to the electrolyte imbalance.

摘要

一匹在出生后两周内被诊断出患有原发性严重联合免疫缺陷的马驹,与其母马一起处于半隔离状态饲养。这匹马驹接受了持续的预防性抗生素治疗、来自用马腺病毒疫苗高度免疫的同胞的血浆,以及强化的一般护理。基于红细胞交叉配型和混合淋巴细胞反应,选择了一匹全同胞雌性作为骨髓供体。在35日龄和73日龄进行两次骨髓输注前给予了环磷酰胺。为预防移植物抗宿主病,这匹马驹接受了甲氨蝶呤治疗。未实现造血重建,也没有移植物抗宿主病的迹象。这匹马驹在第二次骨髓移植后四天,即77日龄时突然死亡。它临床上一直没有任何危及生命的传染病,尸检证实其显著没有传染病。最显著的尸检发现是双侧肾病以及心肌变性和纤维化。死亡的可能原因是电解质失衡,尤其是低钾血症,继而影响了心肌。细胞毒性药物,尤其是环磷酰胺引起的肾毒性,可能导致了电解质失衡。

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