Abstract
Omenn syndrome should be suspected at an early age upon recognition of characteristic phenotypic manifestations: erythroderma, desquamation of the skin, enlargement of the lymph nodes, diarrhea, eosinophilia, and hyper-IgE
In our case autoimmunity manifested before recurrent infections developed, complicating the diagnosis, hence improvement was achieved by using immunosuppressive agents
Non-X-ray treated plasma transfusion deteriorated the patient’s condition, as well as the prognosis of future hematopoietic stem-cell transplant, due to GVHD-like reaction as a result of donor T lymphocytes
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Bondarenko, A., Chernyshova, L. (2019). Widespread Erythema and Skin Desquamation. In: Rezaei, N. (eds) Pediatric Immunology. Springer, Cham. https://doi.org/10.1007/978-3-030-21262-9_33
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DOI: https://doi.org/10.1007/978-3-030-21262-9_33
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