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Primary CNS lymphoma after CLIPPERS: a case series
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  • Published on:
    The link between CLIPPERS and lymphoma might be not fortuitous
    • Guillaume Taieb, Neurologist Department of Neurology, CHU Montpellier, Hopital Guy de Chauliac, 80 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France
    • Other Contributors:
      • Clarisse Carra-Dalliere, Neurologist
      • Xavier Ayrignac, Neurologist
      • Pierre Labauge, Neurologist
      • Geneviève de Saint Basile, Immunologist

    We read with great interest the article written by Sofia Doubrovinskaia and colleagues, entitled“Primary CNS lymphoma after CLIPPERS: A case series.”1
    CLIPPERS mimics are numerous (e.g. primary angiitis of the central nervous system, autoimmune gliopathies [related to anti-myelin oligodendrocyte glycoprotein or anti-glial fibrillary acidic protein antibodies], Erdheim-Chester disease, systemic lymphoma, and primary central nervous system lymphoma [PCNSL]).2
    Among these mimics, only patients who eventually developed a PCNSL (EBV-driven or not) fulfilled initially all CIPPERS criteria, including histological features on brain biopsy (i.e. definite CLIPPERS). In this subset of patients, a question arises: is CLIPPERS a prelymphoma state or a paralymphomatous immune response?
    1) Some clues suggest that paralymphomatous immune response seems to be the most likely hypothesis.
    1.1) In all reported patients who eventually developed PCNSL, histological findings at the first brainstem attack fulfilled histological criterion for CLIPPERS (i.e. characteristic perivascular lymphohistiocytic infiltrates with predominance of T4-cells) with no evidence of lymphoma (i.e. atypical or large B-cells and absence of EBV in B-cells).
    1.2) In these patients, while enhancing lesions predominated initially in the pons, as usually described in CLIPPERS, some of them developed PCNSL remote from the brainstem (i.e. periventricular regions).
    1.3) Besides PCNSL, defin...

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    Conflict of Interest:
    None declared.