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Korean J Pediatr. 2015 Sep;58(9):358-361. English. Case Report. https://doi.org/10.3345/kjp.2015.58.9.358
Ju HY , Hong CR , Kim SJ , Lee JW , Kim H , Kang HJ , Park KD , Shin HY , Chae JH , Phi JH , Cheon JE , Park SH , Ahn HS .
Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea. hyshin@snu.ac.kr
Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul, Korea.
Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Department of Radiology, Institute of Radiation Medicine, Seoul National University College of Medicine, Seoul, Korea.
Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
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.

Copyright © 2019. Korean Association of Medical Journal Editors.