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Comment & Response
September 2013

Should CLIPPERS Be Considered a Prelymphoma State or a New Inflammatory Disease?

Author Affiliations
  • 1Department of Neurology, CHU Nîmes, Hôpital Caremeau, Place du Pr Debré, 30029 Nîmes, Cedex, France
JAMA Neurol. 2013;70(9):1200-1201. doi:10.1001/jamaneurol.2013.3652

To the Editor We read with interest the clinical observation titled “Fatal B-cell lymphoma following chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids [CLIPPERS].”1 The patient presented with steroid-responsive brainstem attacks compatible with CLIPPERS. Biopsy findings showed characteristic perivascular lymphohistiocytic infiltrates with T-lymphocyte predominance. During the third attack, magnetic resonance imaging revealed an atypical enhancing pontine lesion. Treatment with steroids and rituximab led to slight transient clinical improvement, followed by rapidly progressive worsening with brainstem herniation and death. Also during the third attack, new biopsy findings disclosed large atypical B lymphocytes positive for Epstein-Barr virus (EBV)–encoded RNA, leading to a final diagnosis of lymphomatoid granulomatosis (LYG).

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