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A case of progressive multifocal leukoencephalopathy under dimethyl fumarate treatment without severe lymphopenia or

Martin Diebold1, Valerian Altersberger1, Bernhard F Décard1

  • 1Neurologic Clinic and Policlinic, Departments of Medicine, Biomedicine and Clinical Research, University Hospital Basel, University of Basel, Basel, Switzerland.

Multiple Sclerosis (Houndmills, Basingstoke, England)
|June 19, 2019
PubMed
Abstract

Insights

Progressive multifocal leukoencephalopathy (PML) can occur in multiple sclerosis patients on dimethyl fumarate (DMF) even without typical risk factors like advanced age or severe lymphopenia. Vigilance for PML is crucial for all patients on DMF.

Area of Science:

  • Neuroimmunology
  • Demyelinating Diseases

Background:

  • Dimethyl fumarate (DMF) is a disease-modifying therapy for multiple sclerosis (MS).
  • Progressive multifocal leukoencephalopathy (PML) is a rare but serious adverse event associated with DMF treatment.
  • Current risk stratification for DMF-associated PML relies on age >50 years and lymphocyte counts <500 cells/μL.

Observation:

  • A case report details a 39-year-old female with MS treated with DMF who developed PML.
  • This patient presented with oligosymptomatic PML.
  • She did not exhibit severe lymphopenia (lymphocyte counts consistently >800 cells/μL) or signs of immunosenescence.

Findings:

  • The patient's presentation challenges established risk stratification criteria for DMF-associated PML.
  • This case highlights that PML can occur in younger MS patients with preserved lymphocyte counts.
  • Severe CD8-lymphopenia is noted as a common feature in previously reported DMF-associated PML cases.

Implications:

  • Vigilance for PML is recommended for all persons with MS (pwMS) receiving DMF, irrespective of age or lymphocyte count.
  • The findings suggest that current risk stratification models may not identify all individuals at risk for PML.
  • Further research may be needed to refine PML risk assessment in patients treated with DMF.
Keywords:
Dimethyl fumaratelymphopeniamultiple sclerosisprogressive multifocal leukoencephalopathyrisk stratification

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