Hemophagocytic lymphohistiocytosis diagnosed by brain biopsy.
10.3345/kjp.2015.58.9.358
- Author:
Hee Young JU
1
;
Che Ry HONG
;
Sung Jin KIM
;
Ji Won LEE
;
Hyery KIM
;
Hyoung Jin KANG
;
Kyung Duk PARK
;
Hee Young SHIN
;
Jong Hee CHAE
;
Ji Hoon PHI
;
Jung Eun CHEON
;
Sung Hye PARK
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea. hyshin@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Hemophagocytic lymphohistiocytosis;
Central nervous system;
Brain
- MeSH:
Biopsy*;
Bone Marrow;
Brain Diseases;
Brain*;
Central Nervous System;
Cerebrospinal Fluid;
Diagnosis;
Fever;
Humans;
Jaundice;
Leukocytosis;
Liver;
Lymph Nodes;
Lymphohistiocytosis, Hemophagocytic*;
Pathology;
Splenomegaly
- From:Korean Journal of Pediatrics
2015;58(9):358-361
- CountryRepublic of Korea
- Language:English
-
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.
