Chronic recurrent demyelinating encephalomyelitis associated with hemophagocytic lymphohistiocytosis.
A man aged 31 with remittent fever, leukopenia, polyuria, and splenomegaly in addition to chronic and recurrent episodes of neurologic abnormalities was examined pathologically. A chronic type of perivenous encephalomyelitis was found in the CNS, and chronic inflammatory lesions were noticed in the infundibulo-hypophyseal system and also in the peripheral nerves. Moreover, hemophagocytic lymphohistiocytosis predominated in the reticuloendothelial system. This seemed to be the first adult case of demyelinating disease with hemophagocytic lymphohistiocytosis.
D000328:Adult; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000473:pathology*; D003711:Demyelinating Diseases / Q000473:pathology*; D004679:Encephalomyelitis / Q000473:pathology*; D005267:Femoral Nerve / Q000473:pathology; D006529:Hepatomegaly / Q000473:pathology; D006644:Histiocytes / Q000648:ultrastructure; D006801:Humans; D008206:Lymphatic Diseases / Q000473:pathology*; D008218:Lymphocytosis / Q000473:pathology*; D008264:Macrophages / Q000648:ultrastructure; D008297:Male; D009413:Nerve Fibers, Myelinated / Q000648:ultrastructure; D010587:Phagocytosis; D010904:Pituitary Gland, Posterior / Q000473:pathology; D012584:Sciatic Nerve / Q000473:pathology; D013163:Splenomegaly / Q000473:pathology