title: Granulomatous amebic encephalitis: an under-recognized cause of infectious mortality after hematopoietic stem cell transplantation.
abstract: Granulomatous amebic encephalitis (GAE) is a rare, nearly always fatal form of encephalitis that occurs mostly in the setting of immune compromise or chronic disease. The prevalence and clinical characteristics of this Acanthamoeba infection in hematopoietic stem cell transplant (HSCT) recipients are not well described. We present an HSCT patient in whom the diagnosis of GAE was made at autopsy. A systematic review of previously reported cases is provided to highlight the clinical presentation and early diagnostic features of GAE in HSCT recipients. Amebic infection usually initially involves the skin or lungs over a period of months, and becomes rapidly fatal once it crosses the blood-brain barrier. GAE is usually discovered postmortem owing to lack of awareness of this deadly infection and delay in diagnosis. Subacute presentation of multiple recurrent panniculitis-like subcutaneous nodules associated with eosinophilia and a history of chronic rhinitis or sinusitis warrant investigation for a possible amebic infection. Prolonged corticosteroid use and a recent exposure to unhygienic water are potential risk factors for GAE. Successful outcomes may be achieved with early intensive treatment using a combination of effective drugs.
authors.affiliation: Marlene and Stewart Greenebaum Cancer Center, University of Maryland, Baltimore, Maryland 21201, USA. gakpek@umm.edu
authors.forename: G
authors.initials: G
authors.lastname: Akpek
authors.forename: A
authors.initials: A
authors.lastname: Uslu
authors.forename: T
authors.initials: T
authors.lastname: Huebner
authors.forename: A
authors.initials: A
authors.lastname: Taner
authors.forename: A P
authors.initials: AP
authors.lastname: Rapoport
authors.forename: I
authors.initials: I
authors.lastname: Gojo
authors.forename: Y T
authors.initials: YT
authors.lastname: Akpolat
authors.forename: O
authors.initials: O
authors.lastname: Ioffe
authors.forename: M
authors.initials: M
authors.lastname: Kleinberg
authors.forename: M R
authors.initials: MR
authors.lastname: Baer
mesh_terms: D000048:Acanthamoeba / Q000302:isolation & purification*; D000562:Amebiasis / Q000175:diagnosis* / Q000469:parasitology / Q000473:pathology; D000818:Animals; D001344:Autopsy; D001921:Brain / Q000469:parasitology* / Q000473:pathology; D020808:Central Nervous System Protozoal Infections / Q000175:diagnosis / Q000469:parasitology / Q000473:pathology; D004660:Encephalitis / Q000175:diagnosis* / Q000469:parasitology / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006099:Granuloma / Q000175:diagnosis* / Q000469:parasitology / Q000473:pathology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged