2023
DOI: 10.1111/ped.15516
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Rare diseases presenting with hemophagocytic lymphohistiocytosis

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory disorder characterized by hypercytokinemia caused by excessive activation of cytotoxic T cells and macrophages. HLH is caused by a variety of factors and is classified into primary and secondary HLH. Familial HLH (FHL) types 1–5, X‐linked lymphoproliferative syndrome types 1 and 2, and FHL syndrome with hypopigmentation are all examples of primary HLH. Secondary HLH, on the other hand, is linked to infections, malignant tumors, a… Show more

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“…HLH comprises two conditions: primary HLH (pHLH) and secondary HLH (sHLH). pHLH occurs in the presence of an underlying predisposing genetic defect in the cytolytic pathway , whereas sHLH is acquired in the setting of an infectious, malignant, or autoimmune cause without genetic defects[ 2 ].…”
Section: Introductionmentioning
confidence: 99%
“…HLH comprises two conditions: primary HLH (pHLH) and secondary HLH (sHLH). pHLH occurs in the presence of an underlying predisposing genetic defect in the cytolytic pathway , whereas sHLH is acquired in the setting of an infectious, malignant, or autoimmune cause without genetic defects[ 2 ].…”
Section: Introductionmentioning
confidence: 99%