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PML-IRIS — SCE Infectious Diseases MCQ

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HardImmunocompromised HostPML-IRISSCE Infectious Diseases

A 55-year-old woman on Rituximab for follicular lymphoma develops progressive multifocal leukoencephalopathy (PML). JC virus is detected in CSF. What specific complication can occur after Rituximab is discontinued and immune function recovers?

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Correct answer: EPML-IRIS (immune reconstitution inflammatory syndrome causing paradoxical worsening with cerebral oedema)

After stopping Rituximab, B-cell reconstitution and recovering T-cell responses can trigger PML-IRIS: a paradoxical inflammatory worsening of PML with cerebral oedema, contrast enhancement on MRI, and clinical deterioration. PML-IRIS can be fatal. Management includes high-dose corticosteroids (Dexamethasone or Methylprednisolone) to control the inflammatory component. The timing of IRIS typically coincides with evidence of immune reconstitution (rising lymphocyte counts, CD4 recovery).

Reference: NICE 2024 – PML; Lancet Neurology 2020 – PML-IRIS