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Hemophagocytic lymphohistiocytosis, abbreviated HLH and also known as hemophagocytic syndrome,[1] is an uncommon haematologic disorder that, typically, clinically manifests as fever, splenomegaly and jaundice, with laboratory findings of lymphocytosis and histiocytosis,[2] and has the pathologic finding of hemophagocytosis.
HLH can arise in a number of settings and is thought to arise from T-cell dysregulation.
It has been associated with:
Hyperproduction of cytokines, including interferon-? and tumor necrosis factor-a, by EBV-infected T lymphocytes may play a role in the pathogenesis of HLH. EBV-associated HLH may mimic T-cell lymphoma and is treated with cytotoxic chemotherapy. In contrast, hemophagocytic syndromes associated with nonviral pathogens often respond to treatment of the underlying infection.
HLH comprises familial (primary) hemophagocytic lymphohistiocytosis (FHL) and secondary HLH (SHLH), both clinically characterized by the features described above, in particular fever, hepatosplenomegaly, and cytopenia.
FHL, an autosomal recessive disorder, is invariably fatal when untreated. It is associated with defective triggering of apoptosis and reduced cytotoxic activity, resulting in a widespread accumulation of T lymphocytes and activated macrophages.
Several single nucleotide polymorphisms increase risk of HLH.[3]
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