Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis.
10.5045/kjh.2012.47.4.273
- Author:
Myung Mi KIM
1
;
Mi Sun YUM
;
Hae Won CHOI
;
Tae Sung KO
;
Ho Joon IM
;
Jong Jin SEO
;
Kyung Nam KOH
Author Information
1. Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. tsko@amc.seoul.kr
- Publication Type:Original Article
- Keywords:
Hemophagocytic lymphohistiocytosis;
Central nervous system involvement;
Neurological manifestations;
Cerebrospinal fluid;
Outcome
- MeSH:
Alanine Transaminase;
Aspartate Aminotransferases;
Atrophy;
Central Nervous System;
Central Nervous System Diseases;
Consciousness;
Deglutition Disorders;
Dysarthria;
Facial Paralysis;
Ferritins;
Hemorrhage;
Humans;
Leukocytes;
Lymphohistiocytosis, Hemophagocytic;
Magnetic Resonance Imaging;
Neuroimaging;
Neurologic Manifestations;
Organothiophosphorus Compounds;
Prognosis;
Retrospective Studies;
Seizures
- From:Korean Journal of Hematology
2012;47(4):273-280
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
