CNS Involvement in HLH (CNS-HLH)

Hemophagocytic lymphohistiocytosis: what's new in old diagnostic and clinical criteria?

“…It happens that patients without neurological symptoms have changes in imaging studies and vice versa -patients with multiple neurological changes do not always have visible abnormalities in radiological imaging. Less frequent clinical manifestations of hemophagocytic syndrome include: arrhythmia (mainly in the course secondary to Kawasaki disease) or chronic bloody diarrhea (XLP-2), albinism or pseudoalbinism -typical for primary HLH such as Hermansky-Pudlak Syndrome (HPS), Griscelli syndrome (GS II), and Chediak-Higashi syndrome (CHS) [3,[4][5][6].…”

“…During disease monitoring it is important to perform follow-up lumbar punctures, in which signs of disease retreatment are usually seen earlier than on imaging studies. In primary forms of HLH, CSF lesions are more commonly present, with no abnormalities on CNS MR imaging [6,11].…”

“…It should also be kept in mind that abnormalities seen on imaging studies may be a result of chemotherapy. Examples include changes consistent with posterior reversible encephalopathy (PRES), associated with steroid therapy and CSA [6,11].…”