Evaluation of children with haemophagocytic lymphohistiocytosis (HLH) at Red Cross War Memorial Children's Hospital 1991-2010

Master Thesis

2011

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University of Cape Town

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Haemophagocytic Lymphohistiocytosis (HLH) is a rare haematological disorder in children. However, this is probably an underestimation due to the difficulty in diagnosing the disease. HLH is characterized clinically by persistent fevers, organomegaly, cytopaenias and typical biochemical derangements viz. hypertriglyceridaemia, hyperferritinaemia and hypofibrinogenaemia. Other associated findings include decreased natural killer cell (NKC) function and raised soluble CD 25. The exact pathophysiology of HLH is not completely understood but involves a trigger (often an infection) which sets off an uncontrolled inflammatory cascade, characterized by an increase in hyperactivated macrophages and T lymphocytes which leads to increased production of cytokines, alongside reduced cellular cytotoxicity as a result of reduced or absent NKC function.
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