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Abstract: Macrophagic
activation syndrome or lymphohistiocytic hemophagocytosis is characterized by
an uncontrolled and uncontrolled stimulation of the immune system which results
in the infiltration and destruction of multiple organs by cytotoxic cells and
macrophages with hemophagocytosis, and systemic hyper inflammation. Clinically, it
results in fever, organomegaly, weight loss, and biologically in
bi/pancytopenia, cytolysis/cholestasis, hyperferritinemia, and hemostasis
disorders. The histological demonstration of the macrophage with
hemophagocytosis remains the gold standard, whereas it is often absent at the
very beginning, which can complicate the diagnosis. In adults, the clinical
course is lymphohistiocytic hemophagocytosis mild, resolving with treatment of
the etiology in question, with rapidly fatal multiorgan failure. The aetiological
diagnosis remains an essential step in the management since this syndrome is
mainly secondary in adults. DOI: http://dx.doi.org/10.51505/IJMSHR.2021.5104 |
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