| Title: |
106 Inflammatory peripheral neuropathy in haematological malignancies |
| Authors: |
Zirra, Alexandra, Baheerathan, Aravindhan, D’Sa, Shirley, Lunn, Michael |
| Source: |
Journal of Neurology, Neurosurgery, & Psychiatry (JNNP); 2022, Vol. 93 Issue: 6 pA44-A45, 2p |
| Abstract: |
BackgroundWe report the case of a 62-year-old female with demyelinating sensorimotor polyneuropathy in the context of lymphoplasmacytic lymphoma.Case Presentation62-year-old woman presenting with distal lower limb numbness bilaterally, with a 9-year progressive course. Over the next 3 years, foot drop developed with gradual lower and upper limb involvement.Investigations showed raised IgM paraprotein, 20 g/dL. Bone marrow biopsy identified 2-B cell clonal populations with positive MYD88 mutation in keeping with lymphoplasmacytic lymphoma (LPL). Neuro- physiological studies showed very severe length-dependent, sensorimotor neuropathy. She was VEGF/anti-MAG negative, but anti-GM2 positive. Widely-spaced myelin and moderate axonal loss were seen on sural nerve biopsy.She was diagnosed with a progressive demyelinating neuropathy in the context of LPL.Initial IVIG treatment failed. Second line treatment – cyclophosphamide/rituximab/dexamethasone, improved her lower limb weakness. Ibrutinib later stabilised her weakness in the lower but not upper limbs.Plexus nerve biopsy showed general inflammation, excluding direct clonal infiltration and amyloid deposi- tion. This reconfirmed the diagnosis: inflammatory neuropathy driven by a haematological malignancy. She received further treatment with rituximab-bendamustine.ConclusionUnderstanding the pathological mechanism of polyneuropathies in haematological malignan- cies is of paramount importance for choosing the most effective therapy, as illustrated by the above cas8e7.a.zirra@nhs.net |
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