Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Maierhofer, J.
Management of Critically Ill Adult Patients With Hemophagocytic Lymphohistiocytosis
Humanmedizin; [ Diplomarbeit ] Medizinische Universität Graz; 2024. pp. 49
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- Autor*innen der Med Uni Graz:
- Betreuer*innen:
- Hatzl Stefan
- Krause Robert
- Altmetrics:
- Abstract:
- Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome caused by unrestrained activation macrophages and T cells. The acquired form is primarily found in adults and differs from the inborn form in early childhood. Frequent triggers unleashing the cytokine storm of HLH are infections, malignancies and autoimmune diseases. Recently, an increasing number of cases have also been reported in which the occurrence was associated with immunotherapies. In the intensive care unit, HLH is often difficult to distinguish from sepsis due to its similar clinical appearance. In some cases, both are present at the same time. Early diagnosis and initiation of adequate immunosuppressive therapy is essential for the further course and prognosis of HLH. Therefore, the presence of HLH must be considered in critically ill patients with persistent fever and corresponding symptoms (e.g. splenomegaly, neurological abnormalities) or laboratory changes (e.g. elevated ferritin, cytopenia of 2 or 3 cell rows, elevated transaminases). The diagnosis is made using the HLH2004 criteria. The HScore can be used to calculate the probability of the presence of HLH. High-dose corticosteroids are the cornerstone of HLH therapy. Depending on the trigger, etoposide, immunoglobulins, anakinra or other novel drugs are added. The course of the disease depends on the trigger and the response to therapy, as well as the early start of treatment. Overall, the prognosis of HLH is unfavorable despite maximum intensive medical treatment and it is associated with a high mortality rate. With this narrative review, we aim to summarize the most relevant literature for HLH with implication for intensive care therapy.