Research Week Abstract Book - Northern Health
Research Week Abstract Book - Northern Health
Research Week Abstract Book - Northern Health
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Haematology<br />
HaematOLOGY<br />
HIGH-DOSE METHOTREXATE FOR THE TREATMENT OF RELAPseD Csn ERDheim<br />
CHESTER Disease<br />
Ho P and Smith C.<br />
Background<br />
Erdheim-Chester disease (ECD) is a rare non-Langerhan’s histiocytosis with multi-system involvement including central<br />
nervous system (CNS) disease which confers a poorer prognosis. There is no definitive treatment for ECD, though interferonalpha<br />
may be useful for non-CNS disease, if given for more than 3 months.<br />
Case Report<br />
A 60-year old lady with a 5-year history of stable non-CNS ECD presents with 4 days of diplopia and right arm numbness.<br />
Neurological examination revealed a horizontal gaze palsy and right arm paraesthesia. The MRI demonstrated extensive<br />
brainstem/cerebellar lesion but the PET/CT revealed stable systemic disease. CSF analysis showed raised protein (3.12g/L)<br />
but no evidence of infection or malignancy.<br />
During the first 72-hour period, the patient developed dysarthria and ataxia, necessitating urgent treatment. Interferon-alpha<br />
was not ideal due to its slow onset of action and poor CNS penetration.<br />
High-dose methotrexate was chosen due to its excellent CNS penetration and known therapeutic effect on CNS lymphoma.<br />
This treatment arrested the rapid progression and led to significant improvement in her speech and ataxia. A post-induction<br />
MRI showed a reduction in the size of the brainstem/cerebellar lesion and CSF protein reduced.<br />
The patient remained stable with ongoing high-dose methotrexate for 4 months, but subsequently developed new right-sided<br />
weakness and an increase in the size of her brainstem lesion. She is currently being treated with interferon-alpha.<br />
Conclusion<br />
We describe a case of CNS relapse of ECD in the setting of well controlled systemic disease. High-dose methotrexate was<br />
an effective initial salvage agent but further systemic treatment (e.g. interferon-alpha) may be necessary for a sustained longterm<br />
response.<br />
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