Systemic Epstein-Barr Virus-Positive T-cell Lymphoproliferative Disease of Childhood Presenting as Hemophagocytic Lymphohistiocytosis with Chromosomal Abnormalities / 임상검사와정도관리
Journal of Laboratory Medicine and Quality Assurance
;
: 210-215, 2014.
Article
in English
| WPRIM
| ID: wpr-60456
ABSTRACT
Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease (EBV+ T-cell LPD) is characterized by a clonal proliferation of T-cells, which may trigger hemophagocytic lymphohistiocytosis (HLH). Chromosomal abnormalities in patients with HLH are usually found in association with underlying malignancies. We report here a case of systemic EBV+ T-cell LPD of childhood initially presenting with HLH. A 19-year-old man was admitted to the hospital with a 2-week history of fever. Laboratory data revealed pancytopenia, hypertriglyceridemia, high ferritin levels, and abnormalities in liver function tests. EBV infection was confirmed by serologic tests and real-time polymerase chain reaction. Examination of the bone marrow showed histiocytic hyperplasia and hemophagocytosis. Further investigation revealed atypical lymphoid cells expressing EBV-encoded RNA, CD3, CD4, and CD8. A chromosomal analysis displayed a complex karyotype. Despite intensive treatment, the patient died 15 days after initial presentation. In conclusion, systemic EBV+ T-cell LPD of childhood presenting with HLH and chromosomal abnormalities may progress rapidly and be fatal. Therefore, a diagnostic workup for chromosomal aberration is essential.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pancytopenia
/
Bone Marrow
/
RNA
/
Serologic Tests
/
Lymphocytes
/
T-Lymphocytes
/
Hypertriglyceridemia
/
Chromosome Aberrations
/
Herpesvirus 4, Human
/
Epstein-Barr Virus Infections
Type of study:
Diagnostic study
Limits:
Humans
Language:
English
Journal:
Journal of Laboratory Medicine and Quality Assurance
Year:
2014
Type:
Article
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