Central nervous system involvement in adults with haemophagocytic lymphohistiocytosis: a single-center study
| Autor: | Yini Wang, Zhao Wang, Xiaojing Liu, Yanfei Han, Guilan Cai |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
Adult
Central Nervous System Male Pathology medicine.medical_specialty Adolescent Central nervous system Irritability Single Center Lymphohistiocytosis Hemophagocytic White matter 03 medical and health sciences Young Adult 0302 clinical medicine Outcome Assessment Health Care medicine Humans Histiocyte Aged Retrospective Studies Hemophagocytic lymphohistiocytosis medicine.diagnostic_test business.industry Meningism Brain Magnetic resonance imaging Hematology General Medicine Middle Aged medicine.disease Magnetic Resonance Imaging Survival Analysis medicine.anatomical_structure 030220 oncology & carcinogenesis Female medicine.symptom business Tomography X-Ray Computed 030217 neurology & neurosurgery |
| Zdroj: | Annals of hematology. 96(8) |
| ISSN: | 1432-0584 |
| Popis: | Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system (CNS). Neurological manifestations of HLH have been recognized in different studies with children, but they remain relatively ill-defined in adults with HLH. From March 2008 to October 2014, 289 adult patients with HLH were admitted to our center. Clinical, radiological, and cerebral spinal fluid (CSF) data of the patients with CNS involvement were reviewed, and a retrospective study in our single-center was carried out. CNS involvement was observed in 29 patients (10%) either in their diagnosis process or during disease course. CNS symptoms included disturbance of consciousness, cranial nerve palsies, seizures, headache, limb paralysis, irritability, meningism, and memory loss. CSF analysis was conducted in 17 patients (59%). Among them, 11 patients (65%) were reported as having abnormal CSF. Neuroradiological studies were performed in 25 patients (86%). Among the 13 cases that underwent CT scan, one patient hemorrhaged. Single or multiple hypodense foci were detected in the other 2 patients. Magnetic resonance imaging (MRI) abnormalities were found in 15 patients, including focal lesions in cortical and adjacent subcortical regions with or without variable nodular or ring contrast-enhancement, multiple lesions in white matter, diffuse white matter signal changes, and meningeal enhancement. Basal ganglia, cerebellum, and brainstem lesions were also observed. CNS involvement could also be found in adult patients with HLH, but not as frequent as it was in children. The clinical manifestations could be diversified. By carrying out rigorous CNS examinations, an early diagnosis could be made and it was of the utmost importance for the prevention of further lesions. |
| Databáze: | OpenAIRE |
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