A girl with severe combined immunodeficiency and pronounced malnutrition from chronic diarrhoea is presented. Immunological reconstitution was attempted by transplantation of bone marrow cells from the HL‐A hemiallogeneic father. An initial transplant failed to induce a permanent take of the graft, whereas a second transplant with an increased cell dose ensured a take, which was followed by reconstitution of cell‐mediated immune functions. Fractionation of the transplanted bone marrow cells apparently led to a delay in development of graft‐versus‐host symptoms. Germ‐free isolation and extensive bacterial decontamination markedly reduced the microbial flora and was highly protective against contaminating microorganisms but failed to eradicate completely one strain of Escherichia coli that had invaded the child before institution of this regimen. During a moderate, delayed graft‐versus‐host reaction this strain caused widespread severe infection, to which the child succumbed 10 weeks after the second transplantation. This child presented some additional features, the most conspicuous being a deficiency of erythrocyte adenosine deaminase.
Determination of the Y chromatin body should rank among the battery of techniques used to assess the presence of grafted bone marrow cells in cases of male donor and female recipient. The method permits early determination of a take, and probably some measurement of its magnitude. This might provide correlation between level of grafted cells and occurrence of various parameters indicating an immune reconstitution and/or an eventual GVH reaction. The technique may be a highly sensitive marker for chimaerism in cases of histocompatibility between donor and recipient of different sexes.
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