Table 1 Symptoms and pathologic... | American Society of Hematology

Table 1

Symptoms and pathologic characteristics of the 5 patients

Case	Age, y/sex	Symptoms	Biopsy	Previous therapy
1	10/M	Fever, skin lesion, lymphadenopathy, hepatosplenomegaly	Skin and lymph node biopsies showed diffuse infiltration of atypical lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD8⁺, CD20⁻, Ki-67⁺ 60%, CD56⁻, CD68⁺, CD30⁻, Gram-B⁻. In situ hybridization was EBER⁺.	Acyclovir for > 6 mo
2	6/M	Fever, skin lesion, lymphadenopathy, hepatosplenomegaly	Skin biopsy showed diffuse infiltration of atypical T lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD20^light+, Gram-B⁻, CD56⁻, TIA-1⁺, CD68⁺. In situ hybridization was EBER⁺.	IFN-α for 10 months; prednisone for ∼ 6 mo
3	14/M	Fever, lymphadenopathy, hepatosplenomegaly	Liver biopsy showed infiltration of atypical T lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD20⁻, CD2⁺, CD56^light+, CD5⁺, CD7⁺, CD8⁺, CD4^light+, Gram-B⁻, Ki-67⁺ 10%-30%, CD79a⁻. In situ hybridization was EBER⁺.	No specific treatment
4	7/M	Fever, parotid gland swelling	Parotid gland biopsy showed infiltration of atypical lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD4⁺, Granzyme B⁺, CD20⁻, CD56⁻, CD8⁻ Ki-67⁺ 20%. In situ hybridization was EBER⁺.	No specific treatment
5	13/M	Fever, lymphadenopathy, hepatosplenomegaly, rhinopolypus	Rhinopolypus biopsy showed infiltration of atypical lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3^+/−, Granzyme B⁺, CD20⁻, CD56⁺, CD8^+/−, Ki-67⁺ 30%. In situ hybridization was EBER⁺.	Intermittent antiviral therapy for ∼ 4 y; etoposide and prednisone for ∼ 1 y

Case	Age, y/sex	Symptoms	Biopsy	Previous therapy
1	10/M	Fever, skin lesion, lymphadenopathy, hepatosplenomegaly	Skin and lymph node biopsies showed diffuse infiltration of atypical lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD8⁺, CD20⁻, Ki-67⁺ 60%, CD56⁻, CD68⁺, CD30⁻, Gram-B⁻. In situ hybridization was EBER⁺.	Acyclovir for > 6 mo
2	6/M	Fever, skin lesion, lymphadenopathy, hepatosplenomegaly	Skin biopsy showed diffuse infiltration of atypical T lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD20^light+, Gram-B⁻, CD56⁻, TIA-1⁺, CD68⁺. In situ hybridization was EBER⁺.	IFN-α for 10 months; prednisone for ∼ 6 mo
3	14/M	Fever, lymphadenopathy, hepatosplenomegaly	Liver biopsy showed infiltration of atypical T lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD20⁻, CD2⁺, CD56^light+, CD5⁺, CD7⁺, CD8⁺, CD4^light+, Gram-B⁻, Ki-67⁺ 10%-30%, CD79a⁻. In situ hybridization was EBER⁺.	No specific treatment
4	7/M	Fever, parotid gland swelling	Parotid gland biopsy showed infiltration of atypical lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3⁺, CD4⁺, Granzyme B⁺, CD20⁻, CD56⁻, CD8⁻ Ki-67⁺ 20%. In situ hybridization was EBER⁺.	No specific treatment
5	13/M	Fever, lymphadenopathy, hepatosplenomegaly, rhinopolypus	Rhinopolypus biopsy showed infiltration of atypical lymphocytes. Immunohistochemistry staining showed tumor cells expressing CD3^+/−, Granzyme B⁺, CD20⁻, CD56⁺, CD8^+/−, Ki-67⁺ 30%. In situ hybridization was EBER⁺.	Intermittent antiviral therapy for ∼ 4 y; etoposide and prednisone for ∼ 1 y

EBER indicates Epstein-Barr virus–encoded early RNAs; TIA-1, T-cell intercellular antigen-1; and IFN-α, interferon-α.