Histiozytose-Syndrome

Zusammenfassung

Die Histiozytosen umfassen eine Gruppe heterogener Erkrankungen ungeklärter Ätiologie mit unterschiedlichem biologischem Verlauf. Langerhans-Zellhistiozytose ist eine nicht-maligne Erkrankung des dentritischen Zellensystems, welche in einer Vielzahl von Organen auftreten kann. Single-System-Erkrankung mit Befall von einem Organsystem hat eine hervorragende Prognose, meist mit geringer Therapieintensität. Multisystemerkrankung mit Befall von Risikoorganen wie Leber, Milz, Lungen oder Knochenmark kann fatal verlaufen und wird mit systemischer Chemotherapie behandelt. Spätfolgen von Krankheitsmanifestationen umfassen Diabetes insipidus und andere Endokrinopathien, orthopädische Probleme, Lungen- /Leberfibrose oder neuropsychologische Probleme.

Hämophagozytische Lymphohistiozytosen (HLH) sind reaktive und häufig tödlich verlaufende Erkrankungen und können durch einen Gendefekt, verschiedene Infektionen, rheumatische Erkrankungen oder Malignome verursacht sein. Es kommt zu einer abnormen ineffektiven Immunreaktion mit Aktivierung von Lymphozyten, Histiozyten und Hyperzytokinämie mit Fieber, Zytopenie, Hepatosplenomegalie, Hypertriglyzeridämie, niedrigem Fibrinogen, Hämophagozytose, Liquorpleozytose und niedriger NK-Zellaktivität. Die Therapie besteht aus immunomodulatorischen Medikamenten, Chemotherapie und bei primärer HLH in einer Knochenmarkstransplantation.

Summary

The Histiocytic syndromes are a group of rare and heterogeneous diseases. Langerhans cell histiocytosis is a non-malignant disorder of dendritic cells that may affect any organ. Single system disease with only one organ affected has an excellent prognosis with only limited therapy. Multisystem disease might take a fatal course when risk organs like lungs, liver, spleen or hematopoetic system are involved and is treated with systemic chemotherapy. Disease related permanent consequences, like diabetes insipidus, endocrinopathies, orthopedic sequelae, lung or liver fibrosis, or neuropsychological problems may occur.

The hemophagocytic lymphohisticytoses (HLH) are reactive and frequently fatal diseases. A primary genetic defect, various infectious agents, rheumatic diseases or malignant processes may lead to an aberrant ineffective immune response with activation of lymphocytes, histiocytes and hypercytokinemia. Typcial findings comprise fever, cytopenias, hepatosplenomegaly, hypertriglyceridemia, low fibrinogen, hemophagocytosis, CSF pleocytosis and low NK-cell activity. Therapy consists of immunomodulatory drugs or chemotherapy and of bone marrow transplantation in primary HLH.

Schlüsselwörter

Langerhans-Zellhistiozytose - hämophagozytische Lymphohistiozytose

Keywords

Langerhans cell histiocytosis - hemophagocytic lymphohistiocytosis

• Literatur

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