Young adult onset systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorders of childhood.
Rinsho Ketsueki
; 56(5): 501-5, 2015 05.
Article
em Ja
| MEDLINE
| ID: mdl-26062673
ABSTRACT
A 20-year-old woman had a fever, pancytopenia, and liver failure, and was suspected to be suffering from chronic active Epstein-Barr virus (EBV) infection, based on the detection of high EBV-DNA and EBV antibody titers at another hospital. At our institution one month later, clinical manifestations had diminished, and antibody titers had decreased but remained elevated relative to normal levels. Four days later, the patient required hospitalization due to fever, liver damage, and cervical lymphadenopathy. Bone marrow examination and lymph node biopsy results showed EBV-positive cytotoxic T-cells that were predominantly CD4-positive. The disease followed a fulminant course and the patient died of multiple organ failure on hospitalization day 11. Because complicated chromosomal aberrations and T-cell receptor gene rearrangements were identified, we diagnosed her as having systemic EBV-positive T-cell lymphoproliferative disorder of childhood. This disease type includes a lymphoproliferative disorder that is associated with chronic active EBV infection. However, it is clinically different from the type following acute EBV infection. We consider distinguishing between these two types to be important for selecting an early diagnostic procedure and the optimal therapy.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfoma de Células T
/
Herpesvirus Humano 4
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Infecções por Vírus Epstein-Barr
Tipo de estudo:
Prognostic_studies
/
Systematic_reviews
Limite:
Adult
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Female
/
Humans
Idioma:
Ja
Revista:
Rinsho Ketsueki
Ano de publicação:
2015
Tipo de documento:
Article