Hemophagocytic Lymphohistiocytosis Gene Mutations in Adult Patients Presenting With CLIPPERS-Like Syndrome.

doi:10.1212/NXI.0000000000000970

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Hemophagocytic Lymphohistiocytosis Gene Mutations in Adult Patients Presenting With CLIPPERS-Like Syndrome.

Neurology(R) neuroimmunology & neuroinflammation - 1 May 2021

Taieb Guillaume, Kaphan Elsa, Duflos Claire, Lebrun-Frénay Christine, Rigau Valérie, Thouvenot Eric, Duhin-Gand Emeline, Lefaucheur Romain, Hoang-Xuan Khe, Coulette Sarah, Ouallet Jean Christophe, Menjot de Champfleur Nicolas, Tranchant Christine, Picard Capucine, Fusaro Mathieu, Sepulveda Fernando E, Labauge Pierre, de Saint Basile Geneviève

Abstract excerpt

OBJECTIVE: To determine whether adult cases of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) may be related to familial hemophagocytic lymphohistiocytosis (HLH) causes, we have screened patients with adult-onset CLIPPERS for mutations in primary HLH-associated genes. METHODS: In our cohort of 36 patients fulfilling the criteria for probable or definite...

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PMID: 33658321
DOI: 10.1212/NXI.0000000000000970
PMCID: PMC7963436

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Source receipt: pubmed, retrieved 7 Sept 2026.

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