familial hemophagocytic lymphohistiocytosis

initialism: FHL
https://doi.org/10.1351/goldbook.13144
Progressive and potentially lethal inflammatory disease caused by an inherited deficiency of perforin.
Note: Large numbers of polyclonal CD8+ T cells accumulate in lymphoid tissue and other organs, and this is associated with activated macrophages that phagocytose blood cells, including erythrocytes and leukocytes.
Source:
PAC, 2012, 84, 1113. (IUPAC glossary of terms used in immunotoxicology (IUPAC Recommendations 2012)) on page 1165 [Terms] [Paper]
Citation: 'familial hemophagocytic lymphohistiocytosis' in IUPAC Compendium of Chemical Terminology, 5th ed. International Union of Pure and Applied Chemistry; 2025. Online version 5.0.0, 2025. https://doi.org/10.1351/goldbook.13144 RIS BibTex EndNote
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