Intracerebral vascular occlusion in familial erythrophagocytic lymphohistiocytosis: a case report of two siblings |
| |
Authors: | T Takano L E Becker |
| |
Institution: | (1) Department of Pathology, The Hospital for Sick Children, 555 University Avenue, M5G 1X8 Toronto, Ontario, Canada;(2) Department of Pathology (Neuropathology), University of Toronton, M5S 1A1 Toronto, Ontario, Canada |
| |
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. |
| |
Keywords: | Familial erythrophagocytic lymphohistiocytosis Vascular occlusion Brain pathology Cytokines |
本文献已被 SpringerLink 等数据库收录! |
|