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Maternal isoimmunisation resulting in combined immunodeficiency and fatal graft-versus-host disease in an infant
Insights
Maternal isoimmunisation during pregnancy caused severe congenital thrombocytopenia and lymphocytopenia in two infants. One infant developed severe combined immunodeficiency (SCID) and died, while the other recovered, suggesting isoimmune lymphocytopenia as a cause for SCID.
Area of Science:
- Immunology
- Perinatology
- Pediatric Hematology
Background:
- Maternal isoimmunisation, the development of antibodies against fetal antigens, can complicate pregnancies.
- Congenital thrombocytopenia and lymphocytopenia are serious conditions affecting newborns.
- Severe combined immunodeficiency (SCID) is a life-threatening disorder of the immune system.
Observation:
- A woman developed antibodies against paternal platelet and lymphocyte antigens over seven pregnancies.
- The last two offspring presented with congenital thrombocytopenia and lymphocytopenia.
- One infant died from SCID with maternal graft-versus-host disease; the other recovered after treatment for presumed sepsis.
Findings:
- Clinical courses strongly suggested an isoimmune process as the cause of both thrombocytopenia and lymphocytopenia.
- Maternal IgG antibody titers against paternal platelets decreased post-pregnancy.
- Maternal serum targeted a non-HLA determinant on paternal lymphocytes, indicating specific antigen involvement.
Implications:
- Isoimmune lymphocytopenia is postulated as a potential mechanism for SCID in infants.
- Intrauterine engraftment of maternal lymphocytes may contribute to immune suppression and SCID.
- This case highlights the importance of considering isoimmune phenomena in congenital immune deficiencies.
Abstract:
Isoimmunisation to platelet and lymphocyte antigens occurred during a woman's seven pregnancies and resulted in congenital thrombocytopenia and lymphocytopenia in the last two offspring. The youngest baby died aged 16 days of severe combined immunodeficiency (SCID) with maternal graft-versus-host disease. The other affected child, however, recovered fully from thrombocytopenia and lymphocytopenia after exchange transfusion for presumed sepsis. The clinical courses indicated that an isoimmune process had caused both the thrombocytopenia and the lymphocytopenia. In accord with this conclusion, maternal titres of IgG antibodies against paternal platelets fell continuously in serial blood samples taken after the last pregnancy. Cytotoxicity studies of the maternal serum towards lymphocytes from the other siblings and from unrelated donors showed that the paternal antigen involved was a non-HLA determinant. It is postulated that isoimmune lymphocytopenia with subsequent immunological suppression is a potential mechanism for SCID and intrauterine engraftment of maternal lymphocytes.