Familial and acquired hemophagocytic lymphohistiocytosis.
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Familial and acquired hemophagocytic lymphohistiocytosis. / Janka-Schaub, Gritta.
in: ANNU REV MED, Jahrgang 63, 2012, S. 233-246.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Familial and acquired hemophagocytic lymphohistiocytosis.
AU - Janka-Schaub, Gritta
PY - 2012
Y1 - 2012
N2 - Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an uncontrolled and ineffective immune response, triggered in most cases by infectious agents, leads to severe hyperinflammation. Familial forms of HLH (FHL), which are increasingly found also in adolescents and adults, are due to genetic defects leading to impaired function of natural killer cells and cytotoxic T cells. These mutations occur either in the perforin gene or in genes important for the exocytosis of cytotoxic granules. Cytotoxic granules contain perforin and granzymes, which induce apoptosis upon entering (infected) target cells. Additionally, perforin is important for the downregulation of the immune response. Acquired forms of HLH are encountered in association with (usually) viral infections, autoinflammatory/autoimmune diseases, malignant diseases, and acquired immune deficiency states (e.g., after organ transplantation). Treatment of HLH includes immune-suppressive and immune-modulatory agents, cytostatic drugs, and biological response modifiers. For patients with FHL, stem cell transplantation is indicated and can be curative.
AB - Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an uncontrolled and ineffective immune response, triggered in most cases by infectious agents, leads to severe hyperinflammation. Familial forms of HLH (FHL), which are increasingly found also in adolescents and adults, are due to genetic defects leading to impaired function of natural killer cells and cytotoxic T cells. These mutations occur either in the perforin gene or in genes important for the exocytosis of cytotoxic granules. Cytotoxic granules contain perforin and granzymes, which induce apoptosis upon entering (infected) target cells. Additionally, perforin is important for the downregulation of the immune response. Acquired forms of HLH are encountered in association with (usually) viral infections, autoinflammatory/autoimmune diseases, malignant diseases, and acquired immune deficiency states (e.g., after organ transplantation). Treatment of HLH includes immune-suppressive and immune-modulatory agents, cytostatic drugs, and biological response modifiers. For patients with FHL, stem cell transplantation is indicated and can be curative.
KW - Humans
KW - Autoimmune Diseases/complications/immunology
KW - Immunosuppression/adverse effects
KW - Infection/complications/immunology
KW - Killer Cells, Natural/pathology
KW - Lymphohistiocytosis, Hemophagocytic/genetics/immunology/pathology
KW - T-Lymphocytes, Cytotoxic/pathology
KW - Humans
KW - Autoimmune Diseases/complications/immunology
KW - Immunosuppression/adverse effects
KW - Infection/complications/immunology
KW - Killer Cells, Natural/pathology
KW - Lymphohistiocytosis, Hemophagocytic/genetics/immunology/pathology
KW - T-Lymphocytes, Cytotoxic/pathology
M3 - SCORING: Journal article
VL - 63
SP - 233
EP - 246
JO - ANNU REV MED
JF - ANNU REV MED
SN - 0066-4219
ER -
