Systemic Epstein-Barr Virus-Positive T-cell Lymphoproliferative Disease of Childhood Presenting as Hemophagocytic Lymphohistiocytosis with Chromosomal Abnormalities.
10.15263/jlmqa.2014.36.4.210
- Author:
Naery YANG
1
;
Wha Soon CHUNG
;
Yeung Chul MUN
;
Jungwon HUH
Author Information
1. Department of Laboratory Medicine, Ewha Womans University Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Korea. JungWonH@ewha.ac.kr
- Publication Type:Case Report
- Keywords:
Epstein-Barr virus infections;
T-cell lymphoproliferative disease;
Chromosomal analysis;
Chromosome aberrations;
Hemophagocytic lymphohistiocytosis
- MeSH:
Bone Marrow;
Chromosome Aberrations*;
Epstein-Barr Virus Infections;
Ferritins;
Fever;
Herpesvirus 4, Human;
Humans;
Hyperplasia;
Hypertriglyceridemia;
Karyotype;
Liver Function Tests;
Lymphocytes;
Lymphohistiocytosis, Hemophagocytic*;
Pancytopenia;
Real-Time Polymerase Chain Reaction;
RNA;
Serologic Tests;
T-Lymphocytes*;
Young Adult
- From:Journal of Laboratory Medicine and Quality Assurance
2014;36(4):210-215
- CountryRepublic of Korea
- Language:English
-
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.
