Pediatr Blood Cancer: Systematic form lineage of eBV-related T-cell lymphatic disease in children.

!---- The systematic form of eBV-related T-cell lymphatic disease (S-EBV-T-LPD) in children consists of three main forms.EBV-positive bloodthirsty lymphatic tissue cell disease (EBV-HLH), systemic EBV-positive T-cell lymphoma (S-EBV-TCL) and systemic chronic active EBV infection (S-CAEBV).these diseases are rare among children in Western countries.here, we describe eight cases of children in such entities.from 1990 to 2015, we retrospectively identified 8 cases (6 clinical and 2 autopsy) of s-EBV-T-LPD in children.documented clinical pathological parameters, including histomorphology, immunoptype, EBV research, and T-cell receptor gene rearrangement studies.included five women and three male patients, from Hispanic, Asian and Caucasian, with ages ranging from 14 months to 9 years.results showed that fever, swollen liver and spleen, cell reduction, EBV serological abnormalities, EBV virus load is very high is a common manifestation.histological examination results showed that EBV-T cells were immersed, structural distortions were different, cytology was atypical, from no to mild cytology atypical to significant lymphoma and increased tissue haemophilus cells.are characterized by CD4 plus or CD8 plus T cell abnormalities, CD5, CD7 and CD3 dim or disappear, flow cytometers show CD2 and CD45 bright or immuno-grouped show CD5 disappear.TCR gene re-arrangement studies showed that all clinical cases had a single gram of gupto (6/6).treatment included chemotherapy under HLH-94 or HLH-2004, and a bone marrow transplant or not, with poor results.in general, EBV-HLH, S-EBV-TCL, and S-CAEBV exhibited many overlapping characteristics in this large-scale pediatric clinical pathology study of S-EBV-T-LPD in the United States.diagnosis is challenging and the overall effect is poor with current HLH-oriented treatments..