title: Haemophagocytic lymphohistiocytosis associated with coccidiomycosis.
abstract: Haemophagocytic lymphohistiocytosis (HLH) is a rapidly fatal disease caused by dysregulated histiocytes leading to an excessive inflammatory reaction. While genetic forms of HLH exist, the most common form is acquired, frequently associated with infection. Here we report the first case of HLH associated with a coccidiomycosis infection. This patient is a 13-year-old previously healthy boy who presented with a flu-like illness, which rapidly progressed to refractory shock, severe ARDS, multiorgan failure and death despite maximal medical therapy, including broad-spectrum antibiotics to treat well-established causes of acquired HLH. Autopsy findings revealed the diagnosis of HLH in the setting of pulmonary coccidiomycosis. Antifungal therapy should be considered in cases of acquired HLH when the underlying aetiology is not clear.
authors.affiliation: Department of Pediatric Critical Care, Stanford University, Palo Alto, California, USA.
authors.forename: Musaab
authors.initials: M
authors.lastname: Ramsi
authors.affiliation: Department of Pediatric Critical Care, Stanford University, Palo Alto, California, USA.
authors.forename: Cristina
authors.initials: C
authors.lastname: Alvira
authors.affiliation: Department of Pediatric Critical Care, Stanford University, Palo Alto, California, USA.
authors.forename: Prashant
authors.initials: P
authors.lastname: Purohit
authors.affiliation: Department of Pediatric Critical Care, Stanford University, Palo Alto, California, USA.
authors.forename: David
authors.initials: D
authors.lastname: Cornfield
mesh_terms: D000293:Adolescent; D000935:Antifungal Agents / Q000627:therapeutic use; D001344:Autopsy; D003045:Coccidioides; D003047:Coccidioidomycosis / Q000150:complications* / Q000175:diagnosis / Q000382:microbiology; D017809:Fatal Outcome; D006644:Histiocytes / Q000473:pathology*; D006801:Humans; D008168:Lung / Q000382:microbiology / Q000473:pathology*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis / Q000209:etiology*; D008297:Male
chemical_list: D000935:Antifungal Agents