106 Inflammatory peripheral neuropathy in haematological malignancies
| Title: | 106 Inflammatory peripheral neuropathy in haematological malignancies |
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| 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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| Items | – Name: Title Label: Title Group: Ti Data: 106 Inflammatory peripheral neuropathy in haematological malignancies – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Zirra%2C+Alexandra%22">Zirra, Alexandra</searchLink><br /><searchLink fieldCode="AR" term="%22Baheerathan%2C+Aravindhan%22">Baheerathan, Aravindhan</searchLink><br /><searchLink fieldCode="AR" term="%22D’Sa%2C+Shirley%22">D’Sa, Shirley</searchLink><br /><searchLink fieldCode="AR" term="%22Lunn%2C+Michael%22">Lunn, Michael</searchLink> – Name: TitleSource Label: Source Group: Src Data: Journal of Neurology, Neurosurgery, & Psychiatry (JNNP); 2022, Vol. 93 Issue: 6 pA44-A45, 2p – Name: Abstract Label: Abstract Group: Ab Data: 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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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1136/jnnp-2022-ABN.142 Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 2 StartPage: A44 Titles: – TitleFull: 106 Inflammatory peripheral neuropathy in haematological malignancies Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Zirra, Alexandra – PersonEntity: Name: NameFull: Baheerathan, Aravindhan – PersonEntity: Name: NameFull: D’Sa, Shirley – PersonEntity: Name: NameFull: Lunn, Michael IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 01 Text: 2022 Type: published Y: 2022 Identifiers: – Type: issn-print Value: 00223050 – Type: issn-print Value: 1468330X Numbering: – Type: volume Value: 93 – Type: issue Value: 6 Titles: – TitleFull: Journal of Neurology, Neurosurgery, & Psychiatry (JNNP) Type: main |
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