A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis With Clonal Karyotype Abnormality.
- Author:
Kyung Hee KIM
1
;
Sun Hyoung KIM
;
Jin Kyung LEE
;
Yunjung CHO
;
Young Kee KIM
;
Dong Hoon SHIN
;
Kyung A LEE
Author Information
1. Department of Laboratory Medicine, College of Medicine, Korea University, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Hemophagocytic lymphohistiocytosis;
Epstein-Barr virus;
Clonal;
Chromosomal abnor-mality;
Fatal clinical course
- MeSH:
Anti-Bacterial Agents;
Bone Marrow;
Chromosome Aberrations;
Fever;
Genome;
Herpesvirus 4, Human;
Humans;
Hyperplasia;
Karyotype*;
Liver Function Tests;
Lymphohistiocytosis, Hemophagocytic*;
Middle Aged;
Pancytopenia;
Polymerase Chain Reaction;
Prognosis;
Receptors, Antigen, T-Cell;
Sepsis;
Terminal Repeat Sequences
- From:The Korean Journal of Laboratory Medicine
2005;25(2):85-89
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
There have been few reports on chromosomal abnormalities in secondary hemophagocytic lym-phohistiocytosis (HLH) including Epstein-Barr virus-associated HLH (EBV-HLH). Clonality of HLH can be determined by karyotypic analysis, T-cell receptor or IgH rearrangement studies, and EBV genome terminal repeat investigation. Chromosome analysis in EBV-HLH is the most important tool currently available for assessment of case-by-case prognosis. We report a case of fatal EBV-HLH with clonal karyotype abnormality. A 57-year-old man was admitted with persistent high fever and hepatosplenomegaly. Laboratory data revealed pancytopenia, hypofibrinogenemia, coagulation abnormalities, and marked abnormalities of liver function tests. EBV-DNA was detected in the bone marrow by PCR. The bone marrow studies showed mature, benign-looking histiocytic hyperplasia with prominent hemophagocytosis and clonal chromosomal abnormality. Although the patient was treated with immunochemotherapy and antibiotics, he died of disseminated intravascular coagu-lopathy and sepsis. Considering this fatal clinical course, it is important to take intensive therapeutic measures if karyotype abnormality is noted in the treatment of EBV-HLH cases.
