Report of the Histiocyte Society Workshop on “Central Nervous System (CNS) Disease in Langerhans Cell Histiocytosis (LCH)”
✍ Scribed by Grois, N.; Broadbent, V.; Favara, B. E.; D'Angio, G.
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 88 KB
- Volume
- 29
- Category
- Article
- ISSN
- 0098-1532
No coin nor oath required. For personal study only.
✦ Synopsis
Central nervous system (CNS) manifestations of Langerhans cell histiocytosis (LCH) remain among the major enigmas of this puzzling disease. The Histiocyte Society therefore covened a workshop on CNS LCH at the time of its 1996 meeting in Vienna, Austria. Prof. H. Gadner and Dr. N. Grois undertook this formidable assignment, and gathered an outstanding faculty to address the issues. A thorough review of the pathology, diagnostic tests, pathophysiology, available therapies and directions for future clinical and laboratory research was the result, and is summarized in the report that appears below.
Of particular interest in the report is the proposed systematization of CNS disease as visualized by modern magnetic resonance imaging techniques. The system, which is described in detail, is based on 38 LCH children with CNS lesions-a relatively large number-available to the investigators for review and analysis.
Another important point brought out in the report concerns a possible mechanism to explain the otherwise inexplicable, inexorable parenchymal disorders sometimes seen in LCH patients. The authors call attention to the pathogenesis of the progressive dementia seen in such diseases as HIV infection. This is associated with inappropriate activation of glutamate receptors and neuronal cell damage and death. Since CNS LCH of this type is rare in children, those with the syndrome might well be enrolled in current clinical studies of adults with similar processes. This seems a potentially fruitful area of research, since it might not only help in the understanding of the pathophysiology of CNS LCH, but also open pathways to effective therapy. Both are badly neded.
It can be seen from the foregoing and what follows that the goals of the Histiocyte Society were amply met in Vienna in 1996, and that Prof. H. Gadner and Dr. N. Grois fully merited the thanks and the congratulations extended to them at that time for the excellent and illuminating meeting they organized.