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

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