Immunomodulation in an Infant with Familial Hemophagocytic Lymphohistiocytosis Following Chemotherapy
| Autor: | Thomas W. McCloskey, Soo Fang Wang, Sujatha Kosuri, Marianne Frieri, Dominick Sabatino, Gretchen Beck, Paul Cardenas |
|---|---|
| Rok vydání: | 2000 |
| Předmět: |
Pulmonary and Respiratory Medicine
biology business.industry medicine.medical_treatment Lymphocyte Hepatosplenomegaly Transforming growth factor beta Familial Hemophagocytic Lymphohistiocytosis CD38 Cytokine medicine.anatomical_structure Pediatrics Perinatology and Child Health Immunology biology.protein Immunology and Allergy Medicine medicine.symptom Hemophagocytosis business Transforming growth factor |
| Zdroj: | Pediatric Asthma, Allergy & Immunology. 14:239-247 |
| ISSN: | 1557-7767 0883-1874 |
| DOI: | 10.1089/pai.2000.14.239 |
| Popis: | 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... |
| Databáze: | OpenAIRE |
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