Fasth A
Barnmedicinska kliniken, Ostra sjukhuset, Göteborg.
Nord Med. 1995;110(12):316-7.
The designation primary immunodeficiency embraces a multiplicity of diseases of which only the more severe constitute indications for BMT (bone marrow transplantation)--e.g. severe combined immunodeficiency (SCID), Wiskott-Aldrich syndrome, familial haemophagocytic lymphohistiocytosis and malignant osteopetrosis. In cases of immunodeficiency, the outcome of BMT is strongly dependent on the patient's age, clinical status at transplantation and the type of immunodeficiency. In children with SCID who undergo BMT during the first few months of life, lasting cures can be obtained in almost 100 percent of the cases, whereas there is only a 15 percent probability of success if the child is older, infected, cannot undergo cytostatic preconditioning or cannot be given T-cell depleted bone marrow.
原发性免疫缺陷这一名称涵盖了多种疾病，其中只有较为严重的疾病才构成骨髓移植（BMT）的指征，例如严重联合免疫缺陷（SCID）、维斯科特-奥尔德里奇综合征、家族性噬血细胞性淋巴组织细胞增生症和恶性骨硬化症。在免疫缺陷病例中，骨髓移植的结果很大程度上取决于患者的年龄、移植时的临床状况以及免疫缺陷的类型。对于在生命最初几个月接受骨髓移植的SCID患儿，几乎100%的病例都能获得持久治愈，而如果患儿年龄较大、已被感染、无法进行细胞毒性预处理或无法接受去除T细胞的骨髓，则成功的概率仅为15%。
