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Title

Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adult-onset Still's disease and concurrent macrophage activation syndrome

Authors
Gianella, Sara
Schaer, Dominik J.
Schwarz, Urs
Kurrer, Michael
Heppner, Frank L.
Fehr, Jörg
Published in American Journal of Hematology. 2008, vol. 83, no. 5, p. 424-427
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a complex inflammatory disease with multiple diagnostic and therapeutic pitfalls. The congenital form, referred to as familial hemophagocytic lymphohistiocytosis (FHL), is often associated with cerebromeningeal involvement, whereas neurological complications are not characteristic of the adult form of secondary HLH (sHLH). Here we report the case of a 20-year-old woman with adult-onset Still's disease (AOSD), retinal microangiopathy and concurrent macrophage activation syndrome (MAS), in the context of sHLH. Following treatment with etanercept, ibuprofen, methylprednisolone, and phenylbutazone for 3 weeks, MAS deteriorated and fatal cerebral edema occurred within only 24 h. The clinical signs and neuropathological findings are discussed with special emphasis on possible relationships between the aggravation of MAS and therapeutic interventions for AOSD. In conclusion, even the slightest sign of mental decline in a patient with AOSD must be considered central nervous system MAS which can be rapidly fatal.
Keywords AdultBrain Edema/diagnosis/etiology/pathologyConfusion/etiologyDrug Therapy, CombinationEarly DiagnosisFatal OutcomeFemaleHumansIbuprofen/therapeutic useImmunoglobulin G/therapeutic useImmunosuppressive Agents/therapeutic useLymphohistiocytosis, Hemophagocytic/blood/etiology/pathologyMacrophage ActivationMagnetic Resonance ImagingMethylprednisolone/therapeutic usePhenylbutazone/therapeutic useReceptors, Tumor Necrosis Factor/therapeutic useRetinal Diseases/etiologyRetinal Vessels/pathologyStill's Disease, Adult-Onset/complications/drug therapy
Identifiers
PMID: 18183612
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GIANELLA, Sara et al. Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adult-onset Still's disease and concurrent macrophage activation syndrome. In: American Journal of Hematology, 2008, vol. 83, n° 5, p. 424-427. https://archive-ouverte.unige.ch/unige:3370

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Deposited on : 2009-09-23

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