Objective:

Discuss a case Hemophagocytic Lymphohistiocytosis (HLH) in a patient with Glioblastoma Multiforme (GBM)

Background:

A 58-year-old man presented with super refractory status epilepticus requiring intubation and seven antiseizure medications to achieve burst suppression. His history was notable for a right parietal GBM diagnosed eight months prior to presentation status post resection, radiation, and Temozolomide, currently on a blinded, randomized clinical trial. Initial brain MRI showed subtle DWI and FLAIR changes in the right parietal lobe. After weaning antiseizure medications, he remained comatose, though there was no recurrence of seizures. Labs became notable for pancytopenia, hyperbilirubinemia, hypertriglyceridemia, and elevated ferritin, concerning for HLH. Bone marrow biopsy supported this diagnosis. Repeat brain MRI showed new DWI and FLAIR changes in the parietal lobes, pons, and cerebellum, raising the question of neurologic involvement of HLH. He was started on Tocilizumab, Ruxolitinib, and Decadron. CSF obtained two weeks after treatment initiation showed elevated protein without pleocytosis and elevated soluble IL-2 receptor. One month after presentation, he was awake and following commands, but repeat brain MRI was unchanged. 

Design/Methods:

NA

Results:

NA

Conclusions:

HLH is a rare, life-threatening syndrome of abnormal immune activation leading to excessive inflammation and tissue destruction. It can be genetic or sporadic and is associated with disruption in immune homeostasis due to infection, malignancy, or a rheumatologic disorder. There are a few reports of HLH associated with GBM. About one-third of patients with HLH have neurologic symptoms such as ataxia, seizures, or disorders of consciousness, which may be the initial presentation of the disease. Radiographic findings are variable, but may include atrophy, necrosis, increased T2 signal, enhancement, or diffusion restriction in the periventricular white matter or, more rarely, in the deep gray nuclei and brainstem. CSF may show pleocytosis, elevated protein, or hemophagocytosis. It is unknown why this patient developed status epilepticus and HLH.