ISSN:
1432-0533
Keywords:
Familial erythrophagocytic
;
lymphohistiocytosis
;
Vascular occlusion
;
Brain pathology
;
Cytokines
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Neuropathological findings in two siblings with familial erythrophagocytic lymphohistiocytosis (FEL) are reported. Case 1 showed the typical neuropathological findings of FEL with lymphohistiocytic infiltration of the leptomeninges and perivascular spaces. A characteristic erythrophagocytosis was detected in inguinal lymph nodes, lung and bone marrow. Case 2 revealed calcification and necrotic lesions in the brain. In the necrotic areas, parenchymal calcification, vascular medial calcification, and occlusion of many vessels due to subendothelial fibrosis were detected. The areas of necrosis correlated with the distribution of occluded vessels. These changes were most prominent in putamen, internal capsule, thalamus and dentate nucleus. Hypercytokinemia is suspected to be the underlying mechanism for the clinical and laboratory findings in patients with FEL, although the relationship to the vascular pathology is unclear.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00294816
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