Circumvention of Early Graft‐versus‐Host Disease in Hemiallogeneic Bonc Marrow Transplantation in a Case of Severe Combined Immunodeficiency

Abstract

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.

Two Separate Genes Controlling Stimulation in Mixed Lymphocyte Reaction in Man

“…The weak MLC reactivity did not yield a sufficient histoincompatibility to permit expression of severe graft vs host reaction following transplantation (11). MLC testing between approximately 10% of HL-A identical combinations can show this weak MLC-reactivity pattern (24)(25)(26) …”

“…2 The MLR-S determinants X and Y are further defined in Table 3. severe combined immunodeficiency (SCID) and this patient (KJ) has been successfully reconstituted immunologically by a bone-marrow transplant from the maternal uncle J (Family RJ Table 3). The clinical report of this bone-marrow transplantation and the principles for donor-selection are presented elsewhere (10,11). As is shown in Table 5, the patient KJ carries the two HL-A haplotypes (10, W18) and (2,W5).…”

Prolonged Diarrhoea in a Child With Severe Combined Immunodeficiency

“…At 4 months of age she was admitted to the University Clinic of Paediatrics, Copenhagen County Hospital, Gentofte, with pneumonia. She had lymphopenia, negative response of lymphocytes to phytohemagglutinin (PHA) and to allogeneic lymphocytes; with low serum IgG and absent IgA and IgM in serum, thus establishing the diagnosis of CID (10).…”