Abstract Details

Title Hemophagocytic Lymphohistiocytosis with Neurological Involvement

Topic Neurotoxicology

Presentation(s) P06 - (-)

Poster/Presentation
Number 211

Objective

Background BACKGROUND: Hemophagocytic Lymphohistiocytosis (HLH) is a severe immunological condition characterized by fevers, hepatosplenomegaly, pancytopenia, coagulopathy, and organ infiltration by phagocytosing histiocytes. Neurological involvement can be prominent and can include seizures, encephalopathy, ataxia, and cranial nerve palsies among other manifestations. In the early summer of 2012, our institution saw seven cases of HLH. Three of these cases had prominent neurological symptoms and signs with corresponding neuroradiological findings.

Design/Methods DESIGN/METHODS: From March to July of 2012, seven patients with bone marrow proven HLH were treated at our institution. Three of these patients had significant neurological involvement, and will be highlighted here.

Results RESULTS: Neurological manifestations included moderate to severe encephalopathy, seizures including status epilepticus, intracerebral hemorrhage, ataxia, and spastic quadriparesis. Neuroradiological findings included leptomeningeal enhancement, patchy cortical and subcortical T2 signal hyperintensity and enhancement, symmetric basal ganglia T2 signal hyperintensity and enhancement, brainstem T2 signal hyperintensity, and restricted diffusion in the splenium of the corpus callosum. All of the patients at our institution were treated with dexamethasone and etoposide. Of the three patients with neurological involvement, one died, one survived with severe disability, and one survived with moderate disability. Specific details of each case will be presented.

Conclusions CONCLUSIONS: These three cases demonstrate the spectrum of neurological disease that can be seen in HLH. Greater recognition of this rare disorder is important since the neurological manifestations are varied, can be severe, and occur early in the course of the disease.

Authors/Disclosures
Sean Gratton, MD (University Health) PRESENTER	Dr. Gratton has received personal compensation in the range of $0-$499 for serving as a Annual Meeting Planning Committee with Kansas City Clinical Neuroscience Society.
Kenneth J. Shulman, DO (Teva Pharmaceuticals)	No disclosure on file
Carlo S. Tornatore, MD (Medstar Georgetown University Hospital)	Dr. Tornatore has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Serono. Dr. Tornatore has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Tornatore has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Tornatore has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen.

��ɫ��

��ɫ��