Pathogenesis, Diagnostic and Therapeutic Problems in Hemophagocytic Lymphohistiocytosis
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概要
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Hemophagocytic lymophohistiocytosis (HLH) is a hyperinflammatory condition resulting from an uncontrolled ineffective immune response, where proliferation of activated T-lymphocytes and macrophages is caused by dysfunctional cytotoxic T cells. HLH occurs in association with inherited (genetic or familial) diseases or with various non-hereditary diseases, termed as primary or secondary HLH. Among primary HLH, familial hemophagocytic lymphohistiocytosis (FHL) is most common, of which subtypes (FHL2, 3, 4) can now be molecularly diagnosed. The disease develops mostly in infancy except for rare late-onset cases and is fatal if not appropriately treated. Secondary HLH may develop associated with infections, malignancies and autoimmune disorders, the outcome of which varies. In clinical practice, HLH has been recognized as distinct clinical entity over the past 20 years; however, clarification of unidentified genetic defects explaining the pathogenesis in half of FHL cases remains as a challenging problem. This report briefly summarizes the recent progress in this field and emphasizes that the disease heterogeneity needs to be considered in the diagnosis and treatment in the patients with HLH.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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