Neuroinflammatory Disease as an Isolated Manifestation of Hemophagocytic Lymphohistiocytosis.

doi:10.1007/s10875-020-00814-6

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Article

Neuroinflammatory Disease as an Isolated Manifestation of Hemophagocytic Lymphohistiocytosis.

Journal of clinical immunology - 1 Aug 2020

Blincoe Annaliesse, Heeg Maximilian, Campbell Patrick K, Hines Melissa, Khojah Amer, Klein-Gitelman Marisa, Talano Julie-An, Speckmann Carsten, Touzot Fabien, Lankester Arjan, Legger Geertje E, Rivière Jacques G, Garcia-Prat Marina, Alonso Laura, Putti Maria C, Lehmberg Kai, Maier Sarah, El Chazli Yasmine, Elmaksoud Marwa Abd, Astigarraga Itziar, Kurjane Natalja, Bulina Inita, Kenina Viktorija, Bryceson Yenan, Rascon Jelena, Lortie Anne, Goldstein Gal, Booth Claire, Worth Austen, Wassmer Evangeline, Schmitt Erica G, Warren Julia T, Bednarski Jeffrey J, Ali Salah, Chiang Kuang-Yueh, Krueger Joerg, Henry Michael M, Holland Steven M, Marsh Rebecca A, Ehl Stephan, Haddad Elie

Abstract excerpt

Isolated neuroinflammatory disease has been described in case reports of familial hemophagocytic lymphohistiocytosis (FHL), but the clinical spectrum of disease manifestations, response to therapy and prognosis remain poorly defined. We combined an international survey with a literature search to identify FHL patients with (i) initial presentation with isolated neurological symptoms; (ii) absence of cytopenia and...

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PMID: 32638196
DOI: 10.1007/s10875-020-00814-6

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