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- W2024944593 abstract "Reticular dysgenesis (RD), a very rare form of severe combined immunodeficiency (SCID) of undefined etiology, is characterized by severe agranulocytosis with preserved erythroid and megakaryocytic lineages, lymphopenia/athymia, sensorineural hearing loss, and dermatitis. Unlike other SCID forms, the rarity and early lethality of RD preclude rigorous analyses. Limited RD reports lack serial pre-transplant data and most describe circulating T-cell predominance of suspected or verified maternal (M) origin. We identified an unusual RD lymphocyte phenotype with also discordance between the infant's peripheral blood and skin and, M cells composed lymphoid populations within the skin but not the infant's blood. Serial Lymphocyte Flow Cytometry/Lymphoproliferation; Imaging Immunohistochemistry; Chimeric/Genetic Analyses; Breast Milk Irradiation. Newborn-breast-fed female non-consanguineous product without siblings/familial immunodeficiencies; omphalitis; deaf; evanescent dry rash; vacant anterior mediastinum; blood - ALC 3,100; ANC 60 (untreatable); 84%CD19; 6%CD3; 9%CD56; 0%CD45RA; no IgA/IgM; IgG present; absent lymphoproliferation; bone marrow - rare myeloid precursors; Neonate-Infant-non-surgical necrotizing enterocolitis; desquamating dermatitis/alopecia; normal liver; blood - ALC 370; ANC 20; 93%CD19; 4%CD3; 4%CD56; 0%M cells; skin - 100%T cells; 29%M DNA; irradiated/expressed breast milk. Serial RD lymphocyte analyses confirmed a peripheral B-cell preponderant phenotype (100% infant; 0% M) with inverse majority lymphocyte distributions between infant's blood (B+) and skin (T+), and skin cells of M origin. These data plus graft-versus-host disease prompted feeding change from on-breast to expressed and irradiated breast milk during imminent transplant preparations. M cells in RD-SCID lineage phenotype, immunosuppression, and pathogenesis are implicated without yet defined roles based on source, distribution and causal mechanisms." @default.
- W2024944593 created "2016-06-24" @default.
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- W2024944593 date "2008-02-01" @default.
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- W2024944593 title "Serial Lymphocyte Expressions with Unusual and Discordant Distribution of Cellular Populations in the Periphery and Skin in Pre-Transplant Reticular Dysgenesis" @default.
- W2024944593 doi "https://doi.org/10.1016/j.jaci.2007.12.601" @default.
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