Reference Age/ sex Jones et al. two/M [11] Race Case description Unspecified Fever, generalized erythematous skin eruption, hepatosplenomegaly, pancytopenia, hypoplastic bone marrow, pulmonary infiltrates Unspecified Fever, generalized lymphadenopathy, hepatosplenomegaly, pancytopenia, diarrhea, gastric pain Unspecified Gluten enteropathy for 19 years; fever, persistent diarrhea, nodular erythematous skin lesion Craig et al. 20 [15] months/ F Quintanilla- 37/M Martinez et al. [6] Gaillard et seven/F Unspecified Infectious acute mononucleosis, persistent high- al. [13] grade fever, weight loss, adenopathy, necrotizing skin lesions and VAHS Unspecified Fever, generalized erythematous skin eruption, hepatosplenomegaly Fever, mental status of one week duration, hepatosplenomegaly, pancytopenia, jaundice Symptoms of viral upper respiratory illness, hepatosplenomegaly, pancytopenia, jaundice Fever, night sweats, weight loss, hepatosplenomegaly, pancytopenia, jaundice, generalized lymphadenopathy Fever weight loss, hepatosplenomegaly, jaundice Fever, skin rash, hepatosplenomegaly, pancytopenia IM at eight years old, followed by CAEBV. At 14 years old developed hepatosplenomegaly and hemophagocytic syndrome. Tabanelli et al. Journal of Medical Case Reports 2011, 5:218 http://www.jmedicalcasereports.com/content/5/1/218 Page 4 of 5 Table 1 Cases of systemic Epstein-Barr virus positive (EBV+) T cell lymphoproliferative disease (LPD) of childhood described in Western countries 31/F 55/M 17/M 23/M 22/F White Native American Asian Native American 27 Native months/ American M 15/F White Time to Histopathological lymphoma features TCR status EBV status six years one year one year four months - - - - - - - - - - Pulmonary large cell TCR-b EBV+, lymphoma (phenotype: rearranged clonal CD4+, HLA-DR+) Lymphoblastic TCR-b g EBV+, lymphoma (phenotype: rearranged clonal CD4+, HLA-DR+) Peripheral T cell lymphoma (phenotype: UCHL1+) - Fulminant EBV+ T cell TCR-b g LPD (phenotype: CD8+) rearranged EBV+ EBV+ TCR-b EBV+, rearranged clonal T cell lymphoma NOS (phenotype: not interpretable) Fulminant EBV+ T cell TCR-g EBV+, LPD (phenotype: CD4+, rearranged clonal TIA1+) Fulminant EBV+ T cell TCR-g EBV+, LPD (phenotype: CD8+, rearranged clonal TIA1+) Fulminant EBV+ T cell TCR-g EBV+, LPD (phenotype: CD4+, rearranged clonal CD8+, TIA1+) Fulminant EBV+ T cell LPD (phenotype: CD4+, TIA1+) Polyclonal EBV+, clonal Fulminant EBV+ T cell TCR-g EBV+, LPD (phenotype: CD8+, rearranged clonal TIA1+) Fulminant EBV+ T cell TCR-g EBV+, LPD (phenotype: CD4+, rearranged clonal CD8+, TIA1+) Fulminant EBV+ T cell LPD (phenotype: not reported) Fulminant EBV+ T cell LPD (phenotype: not reported) Fulminant EBV+ T cell LPD (phenotype: not reported) TCR-b g EBV+, rearranged clonal TCR-b EBV+, rearranged clonal TCR-b EBV+, rearranged biclonal Wick et al. 12/M [14] Unspecified Hemophagocytic syndrome, FIM three/F Unspecified Hemophagocytic syndrome, FIM nine/M Unspecified Hemophagocytic syndrome, FIM CAEBV = chronic active EBV infevtion; FIM = fatal infectious mononucleosis; HLA = human leukocyte antigen; IM = infectious mononucleosis; NOS = not otherwise specified; TCR = T cell receptor; VAHS = virus-associated hemophagocytic syndrome. description of rare disorders is essential for improving current knowledge and possibly future therapeutic approaches. Consent Written informed consent was obtained from the patient for publication of this case report and any accompany- ing images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Acknowledgements This work was supported by Centro Interdipartimentale per la Ricerca sul Cancro ‘G Prodi’, BolognAIL, AIRC (IG4987; IG1007; and 5xMille), RFO (to SAP and PPP), Fondazione Cassa di Risparmio in Bologna, Fondazione della Banca del Monte e Ravenna, Progetto Strategico di Ateneo 2006 (to SAP and PPP). Author details 1Department of Hematology and Oncological Sciences ‘L and A Seràgnoli’, Hematopathology Section, S Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy. 2Hematology, Department of Cellular Biotechnologies and Hematology, ‘Sapienza’ University, Rome, Italy.