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KMID : 1130320150580090358
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Korean Journal of Pediatrics
2015 Volume.58 No. 9 p.358 ~ p.361
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Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy
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Ju Hee-Young
Hong Che-Ry
Kim Sung-Jin
Lee Ji-won
Kim Hye-Ry
Kang Hyoung-Jin
Park Kyung-Duk
Shin Hee-Young
Chae Jong-Hee
Phi Ji-Hoon
Cheon Jung-Eun
Park Sung-Hye
Ahn Hyo-Seop
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Abstract
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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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KEYWORD
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Hemophagocytic lymphohistiocytosis, Central nervous system, Brain
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