Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy

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...

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Bibliographic Details
Published in:Korean journal of pediatrics 2015, Vol.58 (9), p.358-361
Main Authors: Ju, Hee Young, Hong, Che Ry, Kim, Sung Jin, Lee, Ji Won, Kim, Hyery, Kang, Hyoung Jin, Park, Kyung Duk, Shin, Hee Young, Chae, Jong-Hee, Phi, Ji Hoon, Cheon, Jung-Eun, Park, Sung-Hye, Ahn, Hyo Seop
Format: Article
Language:Korean
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Summary: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.
ISSN:1738-1061
2092-7258