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Comment & Response |

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

Guillaume Taieb, MD1; Dimitri Renard, MD1; Pierre Labauge, MD, PhD1
[+] 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.
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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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September 1, 2013
Hjalmar J. De Graaff, BSc; Mike P. Wattjes, MD, PhD; Annemieke J. Rozemuller, MD, PhD; Axel Petzold, MD, PhD; Joep Killestein, MD, PhD
1MS Center Amsterdam, Department of Neurology, VU University Medical Center, Amsterdam, the Netherlands
2MS Center Amsterdam, Department of Radiology, VU University Medical Center, Amsterdam, the Netherlands
3Department of Pathology, VU University Medical Center, Amsterdam, the Netherlands
JAMA Neurol. 2013;70(9):1201. doi:10.1001/jamaneurol.2013.3855.
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