Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis
Myung-Mi Kim1, Mi-Sun Yum, Hae-Won Choi
1Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Insights
Central nervous system (CNS) involvement is common in hemophagocytic lymphohistiocytosis (HLH), affecting nearly half of patients. CNS involvement in HLH is associated with poorer outcomes and necessitates intensified chemotherapy.
Area of Science:
- Hematology
- Neurology
- Pediatrics
Background:
- Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome.
- Central nervous system (CNS) involvement is a frequent and serious complication of HLH.
Purpose of the Study:
- To compare clinical characteristics, treatment strategies, and prognosis of HLH patients based on the extent of CNS involvement.
- To identify factors influencing outcomes in HLH patients with CNS disease.
Main Methods:
- Retrospective analysis of 50 HLH patients treated between January 1995 and August 2011.
- CNS involvement defined by neurological symptoms or elevated cerebrospinal fluid (CSF) white blood cell (WBC) counts.
- Evaluation of clinical data, laboratory results, treatment regimens, and patient outcomes.
Main Results:
- 46% of HLH patients (23/50) exhibited CNS involvement, presenting with neurological symptoms or elevated CSF WBC.
- Abnormal brain imaging (MRI) was observed in 12 patients, showing lesions, ventriculomegaly, or leptomeningeal enhancement.
- Patients with CNS involvement had lower ferritin, AST, and ALT levels and a significantly reduced 5-year survival rate compared to those without CNS disease.
Conclusions:
- CNS involvement is a common and critical complication in HLH patients.
- HLH patients with CNS disease experience poorer prognoses.
- Early detection of neurological manifestations and intensified chemotherapy are crucial for improving outcomes in HLH patients with CNS involvement.
Background:
Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement.
Methods:
The clinical manifestations, initial laboratory data, treatment, and outcomes for 50 patients diagnosed with HLH and treated at Asan Medical Center between January 1995 and August 2011 were retrospectively reviewed and analyzed. CNS involvement was defined as the presence of neurological symptoms or an elevated white blood cell (WBC) count in the cerebrospinal fluid (CSF).
Results:
Among these 50 patients, 23 (46%) developed CNS disease. Among patients with CNS disease, 19 had neurological symptoms, including seizures, altered consciousness, facial palsy, dysarthria, and dysphagia. Four patients had elevated CSF WBC counts without neurological symptoms. Twelve patients had abnormal brain imaging results, including high signal intensity lesions on T2-weighted magnetic resonance imaging (MRI) findings, ventriculomegaly, hemorrhage, atrophy, and leptomeningeal enhancement. Patients with CNS disease had lower ferritin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels as well as reduced 5-year survival as compared to those without CNS disease.
Conclusion:
CNS involvement is common among patients with HLH. Overall, patients with CNS disease achieve poorer outcomes than patients without CNS involvement. To improve outcomes, physicians must carefully monitor the neurological manifestations in patients with HLH and administer the appropriate course of intensified chemotherapy to patients with CNS disease.
Related Concept Videos
01:01Regulation of Hematopoietic Stem Cells
01:26Encephalitis ll: Pathophysiology
01:28Role of Hematopoietic Growth Factors
Thrombopoietin (TPO), mainly released by the liver,...