Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy

  • Ju, Hee Young (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Hong, Che Ry (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Kim, Sung Jin (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Lee, Ji Won (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Kim, Hyery (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Kang, Hyoung Jin (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Park, Kyung Duk (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Shin, Hee Young (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital) ;
  • Chae, Jong-Hee (Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital) ;
  • Phi, Ji Hoon (Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine) ;
  • Cheon, Jung-Eun (Department of Radiology, Institute of Radiation Medicine, Seoul National University College of Medicine) ;
  • Park, Sung-Hye (Department of Pathology, Seoul National University College of Medicine) ;
  • Ahn, Hyo Seop (Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital)
  • Received : 2013.08.19
  • Accepted : 2014.01.08
  • Published : 2015.09.10
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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.

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References

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