A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis With Clonal Karyotype Abnormality / 대한진단검사의학회지
The Korean Journal of Laboratory Medicine
; : 85-89, 2005.
Article
em Ko
| WPRIM
| ID: wpr-156955
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Pancitopenia
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Prognóstico
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Medula Óssea
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Receptores de Antígenos de Linfócitos T
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Reação em Cadeia da Polimerase
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Aberrações Cromossômicas
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Genoma
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Herpesvirus Humano 4
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Sepse
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Sequências Repetidas Terminais
Tipo de estudo:
Prognostic_studies
Limite:
Humans
Idioma:
Ko
Revista:
The Korean Journal of Laboratory Medicine
Ano de publicação:
2005
Tipo de documento:
Article