Inundate and Deluge-Hemophagocytic Lymphohistiocytosis
Journal Title: Journal of Clinical Immunology & Microbiology - Year 2023, Vol 4, Issue 1
Abstract
Hemophagocytic lymphohistiocytosis is a pathological syndrome constituted of defective Natural Killer (NK) cell or T cell function engendering unchecked secretions of cytokines and end organ damage on account of accompanying immune activation. Hemophagocytic lymphohistiocytosis is categorized into congenital, genetic or primary and acquired or secondary subtypes. Immune activation with consequent hyper-cytokinemia induces histiocytic infiltration within reticuloendothelial organs along with hemophagocytosis of erythrocytes, leukocytes, platelets and precursor cells. Untreated primary and secondary hemophagocytic lymphohistiocytosis is invariably associated with disease associated mortality. However, adoption of contemporary therapeutic protocols document 5 year survival rates of ~ 54%. Familial or primary hemophagocytic lymphohistiocytosis is a progressive, autosomal recessive condition characteristically occurring within young age. The disorder exemplifies decimated NK cell function and frequently detected mutations within perforin gene. Contingent to concordant genetic modifications, the autosomal recessive familial or primary hemophagocytic lymphohistiocytosis demonstrates cogent subtypes denominated as FHL1 to FHL5. Besides, familial hemophagocytic lymphohistiocytosis may be associated with primary immunodeficiency syndromes as Chédiak-Higashi syndrome, Griscelli syndrome type 2, Hermansky-Pudlak syndrome type 2 or X-linked lymphoproliferative disorder subtype 1 and subtype 2 [1,2].
Authors and Affiliations
Anubha Bajaj
Keywords
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- EP ID EP714133
- DOI http://dx.doi.org/10.46889/JCIM.2023.4101
- Views 25
- Downloads 0
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