Chonnam Med J.
2002 Sep;38(3):275-279.
Hemophagocytic Lymphohistiocytosis Presenting with CNS Involvement
- Affiliations
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- 1Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea. hjoonk@chonnam.ac.kr
- 2Department of Diagnostic Radiology, Chonnam National University Medical School, Gwangju, Korea.
- 3Department of Diagnostic Medicine, Chonnam National University Medical School, Gwangju, Korea.
- 4Chonnam National University Research Institute of Medical Sciences, Gwangju, Korea.
Abstract
- Hemophagocytic lymphohistiocytosis (HLH) is an uncommon syndrome characterized by a reactive, systemic proliferation of non-malignant histiocytes through the reticuloendothelial systems. Rarely, the proliferation of phagocytic histiocytes may be seen in brain, lungs, or heart in severe form of HLH, resulting in fatal outcome unless the diagnosis is made early and followed by prompt therapeutic intervention. We report a 24-year-old woman with HLH presenting with abnormal neurologic symptoms and typical imaging findings. Bone marrow aspirate revealed the proliferation of histiocytes and prominent hemophagocytosis. Magnetic resonance imaging of the brain showed diffuse white matter changes throughout the mid-brain, pons, and medullar oblongata. Despite of intensive managements including systemic and intrathecal chemotherapy with immuno-suppressive therapy, she died of adult respiratory distress syndrome.
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