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Haemophagocytic lymphohistiocytosis in adult critical care.


ABSTRACT: Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune dysregulation, characterised by extreme inflammation, fever, cytopaenias and organ dysfunction. HLH can be triggered by conditions such as infection, autoimmune disease and malignancy, among others. Both a familial and a secondary form have been described, the latter being increasingly recognised in adult patients with critical illness. HLH is difficult to diagnose, often under-recognised and carries a high mortality. Patients can present in a very similar fashion to sepsis and the two syndromes can co-exist and overlap, yet HLH requires specific immunosuppressive therapy. HLH should be actively excluded in patients with presumed sepsis who either lack a clear focus of infection or who are not responding to energetic infection management. Elevated serum ferritin is a key biomarker that may indicate the need for further investigations for HLH and can guide treatment. Early diagnosis and a multidisciplinary approach to HLH management may save lives.

SUBMITTER: Bauchmuller K 

PROVIDER: S-EPMC7401439 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature

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Haemophagocytic lymphohistiocytosis in adult critical care.

Bauchmuller Kris K   Manson Jessica J JJ   Tattersall Rachel R   Brown Michael M   McNamara Christopher C   Singer Mervyn M   Brett Stephen J SJ  

Journal of the Intensive Care Society 20200107 3


Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune dysregulation, characterised by extreme inflammation, fever, cytopaenias and organ dysfunction. HLH can be triggered by conditions such as infection, autoimmune disease and malignancy, among others. Both a familial and a secondary form have been described, the latter being increasingly recognised in adult patients with critical illness. HLH is difficult to diagnose, often under-recognised and carries a high mortality. Patie  ...[more]

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