Familial erythrophagocytic lymphohistiocytosis
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- Martin, J.J. & Cras, P. Acta Neuropathol (1985) 66: 140. doi:10.1007/BF00688689
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The neuropathologic study of a 7-monthold female patient affected by familial erythrophagocytic lymphohistiocytosis (FEL) reveals three main features: (1) a lymphohistiocytic leptomeningitis with erythrophagocytosis; (2) perivascular lymphohistiocytic cuffs in the cerebral and cerebellar white matter and, to a lesser extent, in the thalamus, the reticular formation of the brain stem and the griseum pontis; (3) perifocal gliosis and demyelination, especially in the cerebellar white matter.
The lesions remind of the perivenous post-in-fectious encephalomyelitides. Perifocal demyelination has been reported in FEL very seldom. Although its pathogenesis is not known, immune mechanisms could play a role by analogy with perivenous encephalomyelitis.