[Clinical and pathological analysis of three cases of childhood systemic EB virus positive T-cell lymphoproliferative disease].

OBJECTIVE

To explore the clinicopathologic features of systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood (EBV+TLPD).

METHODS

Three cases of EBV+ TLPD of childhood were studied by analyzing the clinical features, morphology, immunophenotypings, EBER-1 in situ hybridization(ISH)and T-cell receptor (TCR)γ gene rearrangement.

RESULTS

The age of onset of the three patients was 2, 7 and 10 years, respectively. They all presented with fever, hepatosplenomegaly and liver failure, accompanied by lymphadenopathy. Skin rash occurred in case 2. Laboratory tests confirmed EBV infection. Lymph node biopsy showed effacement of normal architecture. The residue T zone was significantly expanded, with marked proliferation of arborizing high endothelial venules and prominent infiltration of small to medium-sized atypical lymphocytes. The immunostaining showed cytotoxic T cell phenotypes, e.g. CD3, CD5, T-bet and TIA-1 were all positive, while granzyme B were positive in two cases; CD4 and CD8 were double positive in two cases, but double negative in one case; CD56, CD21and CXCL13 were negative in all the cases. EBER-1 could be detected in all the cases by ISH. Monoclonal rearrangement of TCRγ gene was detected in two cases by polymerase chain reaction (PCR).

CONCLUSION

EBV+ TLPD of childhood is a rare type of peripheral T cell lymphoma. EBV detection by ISH and molecular cloning technique analysis could contribute to the diagnosis. The clinicopathologic characteristics of our cases are consistent with that reported in international literature.