Abstract

ABSTRACT Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive disorder associated with hepatosplenomegaly, cytopenias, lymphohistiocytic accumulation in the reticuloendothelial system and hemophagocytosis. Immunological dysregulation can occur with defective T-and NK-cell function; macrophage activation and cytokine production. Elevated transforming growth factor beta (TGF/β) produced by activated T cells and monocytes can be anti-inflammatory, whereas interleukin-6 (IL-6) induces T-cell proliferation and activation. We report the clinical and immunological evaluation of a 5-month-old Hispanic infant with FHLH. The evaluation included TGF/β, IL-6, nitric oxide (NO), and extended lymphocyte phenotypes, CD3+, CD4+, CD8+, CD28+, CD95+, CD45RA+, CD62L+, HLA-DR+, CD38+, CD69+ using enzyme-linked immunoadsorbent assay (ELISA), Greiss reagent, and 4′ color flow cytometry pre- and postchemotherapy with VP16 @ 65 mg/M2 and dexamethasone over 10 courses of treatment. TGFβ (924.4–4286.8 pg/mL...

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