Central Nervous System Involvement in Hemophagocytic Lymphohistiocytosis: A Single-Center Experience

Clinical features and prognostic factors of adult secondary hemophagocytic syndrome

“…As a result, monitoring of clinical manifestations of the nervous system of patients was extremely important to evaluate the disease as early as possible and improve the survival rate. [34] Jovanovic et al [32] reported 30 children with HPS; 17 patients manifested central nervous system involvement, of which 10 patients died in the end and 3 of the survived patients had moderate-to-severe neurological sequelae. The central nervous system involvement was found to be an important death predictive factor.…”

Hemophagocytic lymphohistiocytosis: A rare cause of recurrent encephalopathy

“…In view of persistent pancytopenia, a bone marrow biopsy was performed. It showed morphological changes in all three cell lines consistent with myelodysplastic syndrome (MDS) with ring 228 CNS involvement in HLH is associated with poor prognosis (3)(4)(5), however, our patient responded well to the HLH treatment and recovered fully after each episode. During his third admission he had a prolonged period of encephalopathy when he had developed transient MDS, in addition to HLH.…”

“…Central nervous system (CNS) involvement in HLH has been reported in 73% of cases at the time of diagnosis (3). It may also develop later during the course of the disease and is associated with poor prognosis (3)(4)(5). Neuropathological studies have shown perivascular infiltration of meninges and brain parenchyma by activated lymphocytes and macrophages with hemophagocytosis and active inflammation.…”

“…This may progress to multifocal necrosis and astrogliosis (6). The neurological manifestations of HLH include varying degrees of encephalopathy, seizures, meningitis, hemiparesis, ataxia and cranial nerve palsy (3)(4)(5). CSF analysis may show raised proteins and or hemophagocytic cells.…”

Hemophagocytic lymphohistiocytosis: A diagnostic conundrum

“…[1415] Neurologic symptoms of HLH include, but are not limited to, irritability, seizures, ataxia, cranial nerve palsies, hemiplegia/tetraplegia, mental status change, and/or encephalitis. [5161718] CSF studies may show elevated protein levels and/or pleocytosis, and hypodense/necrotic areas have been observed on MRI of the brain. [1819]…”