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Reversal of enterocolitis-associated combined immunodeficiency by plasma therapy
Insights
Intensive plasma therapy reversed combined immunodeficiency symptoms in infants with severe diarrhea and malabsorption. This treatment normalized immune function and intestinal health, suggesting a potential therapeutic approach.
Area of Science:
- Pediatric Gastroenterology
- Clinical Immunology
- Nutritional Science
Background:
- Two infants presented with severe diarrhea, malabsorption, and hypoproteinemia.
- Initial diagnosis was combined immunodeficiency syndrome.
- Pre-illness values for serum protein and lymphocytes were normal.
Observation:
- Infants exhibited lymphopenia, diminished B and T cells, cutaneous anergy, low immunoglobulin levels, and poor in vitro lymphocyte proliferation.
- Proctosigmoidoscopy revealed rectal ulcerations, rectal biopsy showed colitis, and small bowel biopsy indicated moderate to severe villus abnormalities.
- Plasma cells were absent, and generalized malabsorption of all nutrients was present.
Findings:
- Treatment with irradiated fresh-frozen plasma (11–20 ml/kg/day for 1–2 months) replaced intestinal protein losses.
- During therapy, diarrhea decreased, biopsy morphology improved, and immunoglobulin levels and T-cell function normalized.
- Following plasma therapy discontinuation, immune function remained normal, stool patterns normalized, and malabsorption reversed.
Implications:
- Intensive plasma therapy may contribute to the reversal of immunodeficiency states in similar pediatric cases.
- This approach offers a potential therapeutic strategy for infants presenting with combined immunodeficiency and severe gastrointestinal symptoms.
- Further investigation into plasma therapy's role in reversing acquired immunodeficiency is warranted.
Abstract:
Two 6-month-old male infants with diarrhea, malabsorption, and hypoproteinemia, who were initially diagnosed as having combined immunodeficiency syndrome, recovered with intensive plasma therapy. Prior to the onset of diarrhea, they had normal serum protein and lymphocyte values. Immunologic features of combined immunodeficiency included lymphopenia, diminished B and T cells, cutaneous anergy, low immunoglobulin levels, and poor lymphocyte proliferative responses in vitro. Prior to therapy, both children had rectal ulcerations by proctosigmoidoscopy, colitis by rectal biopsy, and moderate to severe intestinal villus abnormalities by small bowel biopsy; plasma cells were absent Both had generalized malabsorption of all nutrients. Both infants were given irradiated fresh-frozen plasma for one to two months at 11 to 20 ml/kg/day to replace intestinal protein losses. During this time, diarrhea slowed, biopsy morphology improved, and immunoglobulin levels and T-cell function became normal. After discontinuance of plasma therapy, normal immune function and a normal stool pattern with reversal of malabsorption continued. Since intensive plasma therapy may have contributed to the reversal of the immunodeficiency state, a trial of such therapy is recommended in similar patients.