AJHE2108 - CME/CMLE - Cytomorphologic Features Found in Cerebrospinal Fluid Specimens of Hemophagocytic Lymphohistiocytosis Patients: A Report of 64 Cases-Cytomorphologic Features Found in Cerebrospinal Fluid Specimens of Hemophagocytic Lymphohistiocytosis Patients: A Report of 64 Cases

Cytomorphologic Features Found in Cerebrospinal Fluid Specimens of Hemophagocytic Lymphohistiocytosis Patients: A Report of 64 Cases

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The study focused on analyzing cytomorphologic features in cerebrospinal fluid (CSF) from patients with Hemophagocytic Lymphohistiocytosis (HLH), particularly those with Central Nervous System (CNS) involvement. The research identified five CSF cytomorphologic patterns in HLH patients, including hemophagocytosis and vacuolated macrophages, with hemophagocytosis being more prevalent in children with CNS involvement. However, hemophagocytosis was absent in adult patients with CNS involvement. The study highlighted that CSF cytopathologic features could be associated with an increased risk of CNS-HLH. Notably, the cytomorphologic patterns did not correlate well with CSF white blood cell counts or protein concentration, indicating their limited value in diagnosing CNS-HLH.<br /><br />The research emphasized the importance of early recognition of CNS involvement by HLH due to its critical implications. Treatment strategies for CNS-HLH involve systemic corticosteroids combined with other agents like etoposide and cyclosporine A, with additional intrathecal medications for poor responders or children. The study suggested that CSF cytology, along with clinicoradiologic context, could aid in diagnosing CNS-HLH more accurately, especially in pediatric cases.<br /><br />In conclusion, the study provided valuable insights into the cytomorphologic spectrum of CSF involvement in HLH, shedding light on the specific patterns observed in patients with CNS complications. The findings highlighted the potential of CSF cytology as a diagnostic tool for CNS-HLH, particularly in pediatric cases. Further research and validation of these findings may contribute to improving the diagnosis and management of CNS involvement in patients with HLH.

Keywords

cerebrospinal fluid

CSF

Hemophagocytic Lymphohistiocytosis

HLH

Central Nervous System

CNS involvement

cytomorphologic patterns

hemophagocytosis

vacuolated macrophages

diagnosis