Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe inflammatory disorder marked by abnormal cytotoxic T and natural killer cell activity, resulting in impaired clearance of pathogen, excessive cytokine production, and continued immune system activation. Soluble IL-2 receptor (sIL-2R or sCD25) is typically elevated in HLH and can serve as a marker of disease activity, although its role in the pathophysiology of the disease is unclear. Here we present a case of an adult patient with steroid-dependent HLH who was treated successfully with daclizumab, a monoclonal anti-CD25 antibody, allowing successful withdrawal of steroid therapy without an increase in symptoms.
Copyright 2008 Wiley-Liss, Inc.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adrenal Cortex Hormones / therapeutic use
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Antibodies, Monoclonal / therapeutic use*
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Antibodies, Monoclonal, Humanized
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Biomarkers
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Cyclophosphamide / administration & dosage
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Cyclosporine / therapeutic use
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Daclizumab
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Doxorubicin / administration & dosage
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Ferritins / blood
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Humans
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Immunoglobulin G / therapeutic use*
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Immunosuppressive Agents / therapeutic use*
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Interleukin-2 Receptor alpha Subunit / antagonists & inhibitors*
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Interleukin-2 Receptor alpha Subunit / immunology
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Killer Cells, Natural / immunology
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Lymphohistiocytosis, Hemophagocytic / complications
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Lymphohistiocytosis, Hemophagocytic / drug therapy*
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Lymphoma, Large-Cell, Anaplastic / complications
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Lymphoma, Large-Cell, Anaplastic / drug therapy
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Male
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Middle Aged
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Prednisone / administration & dosage
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Recurrence
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T-Lymphocyte Subsets / chemistry
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T-Lymphocyte Subsets / immunology
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Vincristine / administration & dosage
Substances
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Adrenal Cortex Hormones
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Antibodies, Monoclonal
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Antibodies, Monoclonal, Humanized
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Biomarkers
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Immunoglobulin G
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Immunosuppressive Agents
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Interleukin-2 Receptor alpha Subunit
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Vincristine
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Doxorubicin
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Cyclosporine
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Cyclophosphamide
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Ferritins
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Daclizumab
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Prednisone