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Table 1 Cases of systemic Epstein-Barr virus positive (EBV+) T cell lymphoproliferative disease (LPD) of childhood described in Western countries

From: Systemic Epstein-Barr-virus-positive T cell lymphoproliferative childhood disease in a 22-year-old Caucasian man: A case report and review of the literature

Reference Age/sex Race Case description Time to lymphoma Histopathological features TCR status EBV status
Jones et al. [11] two/M Unspecified Fever, generalized erythematous skin eruption, hepatosplenomegaly, pancytopenia, hypoplastic bone marrow, pulmonary infiltrates six years Pulmonary large cell lymphoma (phenotype: CD4+, HLA-DR+) TCR-β rearranged EBV+, clonal
  31/F Unspecified Fever, generalized lymphadenopathy, hepatosplenomegaly, pancytopenia, diarrhea, gastric pain one year Lymphoblastic lymphoma (phenotype: CD4+, HLA-DR+) TCR-β γ rearranged EBV+, clonal
  55/M Unspecified Gluten enteropathy for 19 years; fever, persistent diarrhea, nodular erythematous skin lesion one year Peripheral T cell lymphoma (phenotype: UCHL1+) - EBV+
Gaillard et al. [13] seven/F Unspecified Infectious acute mononucleosis, persistent high-grade fever, weight loss, adenopathy, necrotizing skin lesions and VAHS four months Fulminant EBV+ T cell LPD (phenotype: CD8+) TCR-β γ rearranged EBV+
Craig et al. [15] 20 months/F Unspecified Fever, generalized erythematous skin eruption, hepatosplenomegaly - T cell lymphoma NOS (phenotype: not interpretable) TCR-β rearranged EBV+, clonal
Quintanilla-Martinez et al. [6] 37/M White Fever, mental status of one week duration, hepatosplenomegaly, pancytopenia, jaundice - Fulminant EBV+ T cell LPD (phenotype: CD4+, TIA1+) TCR-γ rearranged EBV+, clonal
  17/M Native American Symptoms of viral upper respiratory illness, hepatosplenomegaly, pancytopenia, jaundice - Fulminant EBV+ T cell LPD (phenotype: CD8+, TIA1+) TCR-γ rearranged EBV+, clonal
  23/M Asian Fever, night sweats, weight loss, hepatosplenomegaly, pancytopenia, jaundice, generalized lymphadenopathy - Fulminant EBV+ T cell LPD (phenotype: CD4+, CD8+, TIA1+) TCR-γ rearranged EBV+, clonal
  22/F Native American Fever weight loss, hepatosplenomegaly, jaundice - Fulminant EBV+ T cell LPD (phenotype: CD4+, TIA1+) Polyclonal EBV+, clonal
  27 months/M Native American Fever, skin rash, hepatosplenomegaly, pancytopenia - Fulminant EBV+ T cell LPD (phenotype: CD8+, TIA1+) TCR-γ rearranged EBV+, clonal
  15/F White IM at eight years old, followed by CAEBV. At 14 years old developed hepatosplenomegaly and hemophagocytic syndrome. - Fulminant EBV+ T cell LPD (phenotype: CD4+, CD8+, TIA1+) TCR-γ rearranged EBV+, clonal
Wick et al. [14] 12/M Unspecified Hemophagocytic syndrome, FIM - Fulminant EBV+ T cell LPD (phenotype: not reported) TCR-β γ rearranged EBV+, clonal
  three/F Unspecified Hemophagocytic syndrome, FIM - Fulminant EBV+ T cell LPD (phenotype: not reported) TCR-β rearranged EBV+, clonal
  nine/M Unspecified Hemophagocytic syndrome, FIM - Fulminant EBV+ T cell LPD (phenotype: not reported) TCR-β rearranged EBV+, biclonal
  1. 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.