Korean J Lab Med.  2005 Apr;25(2):85-89.

A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis With Clonal Karyotype Abnormality

• Kim KH
• Kim SH
• Lee JK
• Cho Y
• Kim YK
• Shin DH
• Lee KA

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.

Keyword

Hemophagocytic lymphohistiocytosis; Epstein-Barr virus; Clonal; Chromosomal abnor-mality; Fatal clinical course

MeSH Terms

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
Anti-Bacterial Agents
Receptors, Antigen, T-Cell