Systemic EBV Positive T-Cell Lymphoproliferative Disorder of Childhood: A Clinicopathologic Study of 5 Cases
Annisa L Lewis, Paola Cortez, Carl Allen, Andrea Sheehan, Tarek Elghetany, Choladda V Curry. Baylor College of Medicine & Texas Children's Hospital, Houston, TX; Hospital Nacional de Ninos-Universidad de Costa Rica, San Jose, Costa Rica
Background: Systemic EBV positive T-cell lymphoproliferative disease of childhood (S-EBV-T-LPD) represents an extremely rare entity in Western countries. It can occur following acute EBV infection or in severe chronic active EBV infection (SCAEBV). However, the disease is not well-characterized in the pediatric US population.
Design: We searched our database for cases of T-LPD or atypical T-cell proliferation with either positive EBV, documented SCAEBV or EBV-associated hemophagocytic lymphohistiocytosis (HLH).
Results: We identified 5 cases of S-EBV-T-LPD (2 male, 3 female, age 14 months to 5 yrs, ethnicity 3 Hispanic, 1 White, 1 Asian). Of four patients with available clinical data all presented with fever, hepatosplenomegaly, & pancytopenia – 3 with acute onset viral illness, consistent with primary acute EBV infection, & HLH. One had documented SCAEBV for 7 years. Histologically, three showed distinct T-LPD with extensive infiltrates of lymph node or liver with atypical medium-sized lymphoid cells - 2 also demonstrated prominent hemophagocytosis with necrosis and apoptosis. Two cases showed subtle T-cell infiltrates - one with mild sinusoidal & portal hepatic infiltrates & one with mild marrow lymphoid infiltrates. One case had T-cell lymphoma and EBV associated HLH with incomplete immunohistochemical profile. The other four cases were CD3+, EBER+, LMP-1-. Two were predominantly CD4+, one was CD8+ & two had mixed CD4+ & CD8+ cells. T-cell clonality by PCR was positive in 3 of 3 cases. Of 4 cases with follow up, two received chemotherapy & died at 29 days & 5 months while awaiting bone marrow transplant (BMT). Two had chemotherapy and BMT. One was alive without disease at 15 yrs and one had progressive disease for 7 years and died 8 months after development of aggressive T-LPD.
Conclusions: To the best of our knowledge, this is the largest series of pediatric S-EBV-T-LPD in the US. S-EBV-T-LPD is characterized by fever, pancytopenia, hepatosplenomegaly, and findings consistent with HLH. It is a fatal disease, though rapid diagnosis and BMT may improve outcome. The diagnosis should be considered when EBER+ T-cells with either extensive or subtle infiltrates are identified in the appropriate clinical setting. T-cell clonality by PCR may be helpful, especially as part of early evaluation of patients with suspected disease.
Monday, March 4, 2013 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 218, Monday Morning