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Familial erythrophagocytic lymphohistiocytosis. A neuropathologic study.

Abstract
The neuropathologic study of a 7-month-old 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-infectious 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.
AuthorsJ J Martin, P Cras
JournalActa neuropathologica (Acta Neuropathol) Vol. 66 Issue 2 Pg. 140-4 ( 1985) ISSN: 0001-6322 [Print] GERMANY, WEST
PMID4013668 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Topics
  • Arachnoid (pathology)
  • Cerebellum (pathology)
  • Demyelinating Diseases (pathology)
  • Erythrocytes (pathology)
  • Female
  • Histiocytes (physiology)
  • Humans
  • Infant
  • Lymphatic Diseases (genetics, pathology, physiopathology)
  • Phagocytosis
  • Pia Mater (pathology)

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