title: Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, splenomegaly, jaundice, and pathologic findings of hemophagocytosis in bone marrow or other tissues such as the lymph nodes and liver. Pleocytosis, or the presence of elevated protein levels in cerebrospinal fluid, could be helpful in diagnosing HLH. However, the pathologic diagnosis of the brain is not included in the diagnostic criteria for this condition. In the present report, we describe the case of a patient diagnosed with HLH, in whom the brain pathology, but not the bone marrow pathology, showed hemophagocytosis. As the diagnosis of HLH is difficult in many cases, a high level of suspicion is required. Moreover, the pathologic diagnosis of organs other than the bone marrow, liver, and lymph nodes may be a useful alternative.
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Hee Young
authors.initials: HY
authors.lastname: Ju
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Che Ry
authors.initials: CR
authors.lastname: Hong
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Sung Jin
authors.initials: SJ
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Ji Won
authors.initials: JW
authors.lastname: Lee
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Hyery
authors.initials: H
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Hyoung Jin
authors.initials: HJ
authors.lastname: Kang
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Kyung Duk
authors.initials: KD
authors.lastname: Park
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Hee Young
authors.initials: HY
authors.lastname: Shin
authors.affiliation: Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Jong-Hee
authors.initials: JH
authors.lastname: Chae
authors.affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Ji Hoon
authors.initials: JH
authors.lastname: Phi
authors.affiliation: Department of Radiology, Institute of Radiation Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Jung-Eun
authors.initials: JE
authors.lastname: Cheon
authors.affiliation: Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Sung-Hye
authors.initials: SH
authors.lastname: Park
authors.affiliation: Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea.
authors.forename: Hyo Seop
authors.initials: HS
authors.lastname: Ahn
keywords: Brain; Central nervous system; Hemophagocytic lymphohistiocytosis