Table 4. Review of previously reported... | American Society of Hematology

Table 4.

Review of previously reported cases of T-cell LPD in patients with CAEBV infection

Reference	Age/ sex	Ethnic origin	Time to lymphoma	Clinical presentation	Histopathologic features	Phenotype	EBV status	TCR status	Therapy and outcome
Jones et al²⁶	2/M	NS	6 y	Fever, dyspnea, pulmonary infiltrates	Pulmonary large cell lymphoma	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β R	CHOP: dead at 18 d
	3/F	NS	1 y	Diarrhea, gastric pain, abdominal distension, generalized lymphadenopathy	Systemic involvement by lymphoblastic lymphoma	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β γ R	Steroids, acyclovir; dead at several days
	55/M	NS	1 y	Gluten enteropathy for 19 y; nodular erythematous skin lesions	Skin and systemic inv by peripheral T-cell lymphoma/LYG^4-150	UCHL-1⁺	EBV⁺	ND	Steroids and cyclophosphamide; dead at several months
Kikuta et al²⁹	1/M	Asian	1 y	Fever, generalized lymphadenopathy, hepatosplenomegaly, pneumonitis, coronary aneurysms	NS: Only peripheral blood analyzed	CD4⁺HLA-DR⁺	EBV⁺	ND	No follow-up given
Ishihara et al²⁸	2/M	Asian	8 y	Fever, hepatosplenomegaly, progressive hepatic dysfunction	NS. Peripheral lymphocytosis, only peripheral blood analyzed	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β R	Steroids, acyclovir Dead at 26 d
Bonagura et al³¹	10/F	NS	6 y	Generalized lymphadenopathy, ascites, maxillary and ethmoid sinus infiltration & frontal lobe lesions by CT scans	Nodal T-cell lymphoma, NOS	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β R	Steroids, acyclovir Dead shortly after
Tanaka et al³²	2/M	Asian	9 y	Multiple nodular lung infiltrates, hemophysis	Pulmonary large cell lymphoma, angiocentric	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β G	Antiviral agents and interferon Dead shortly after
Kanegane et al³⁰	2/F	Asian	2 y	Generalized lymphadenopathy	Nodal T-cell lymphoma, NOS	CD45RO⁺, CD4⁺	EBV⁺, clonal	TCR-β,γ R	Vincristine and prednisone Clinical remission
	11/M	Asian	5 mo	High fever, hepatosplenomegaly, parotid swelling	Nodal T-cell lymphoma, NOS	CD45RO⁺, CD4⁺	EBV⁺, clonal	TCR-β,γ R	α-interferon, cyclosporin A, etoposide, steroids Dead at 2 y and 4 mo
Ohshima et al²⁷	52/M	Asian	5 y	Lymphadenopathy, hepatosplenomegaly	Nodal T-cell lymphoma, NOS	ND	EBV⁺	ND	Dead, time unspecified
	12/F	Asian	11 mo	Lymphadenopathy, hepatosplenomegaly	NK leukemia	CD56⁺	EBV⁺, clonal	TCR-β,γ G	Dead, time unspecified
	23/F	Asian	6 mo	Hepatosplenomegaly	NK leukemia	CD56⁺	EBV⁺, clonal	TCR-β,γ G	Dead, time unspecified
Kawaguchi et al⁴¹	13/F	Asian	20 mo	Hepatosplenomegaly fever, pancytopenia	Bone marrow lymphocytosis	CD45RO⁺, TCRβ⁺	EBV⁺, clonal	TCR-β,γ G	Dead at 20 months

Reference	Age/ sex	Ethnic origin	Time to lymphoma	Clinical presentation	Histopathologic features	Phenotype	EBV status	TCR status	Therapy and outcome
Jones et al²⁶	2/M	NS	6 y	Fever, dyspnea, pulmonary infiltrates	Pulmonary large cell lymphoma	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β R	CHOP: dead at 18 d
	3/F	NS	1 y	Diarrhea, gastric pain, abdominal distension, generalized lymphadenopathy	Systemic involvement by lymphoblastic lymphoma	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β γ R	Steroids, acyclovir; dead at several days
	55/M	NS	1 y	Gluten enteropathy for 19 y; nodular erythematous skin lesions	Skin and systemic inv by peripheral T-cell lymphoma/LYG^4-150	UCHL-1⁺	EBV⁺	ND	Steroids and cyclophosphamide; dead at several months
Kikuta et al²⁹	1/M	Asian	1 y	Fever, generalized lymphadenopathy, hepatosplenomegaly, pneumonitis, coronary aneurysms	NS: Only peripheral blood analyzed	CD4⁺HLA-DR⁺	EBV⁺	ND	No follow-up given
Ishihara et al²⁸	2/M	Asian	8 y	Fever, hepatosplenomegaly, progressive hepatic dysfunction	NS. Peripheral lymphocytosis, only peripheral blood analyzed	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β R	Steroids, acyclovir Dead at 26 d
Bonagura et al³¹	10/F	NS	6 y	Generalized lymphadenopathy, ascites, maxillary and ethmoid sinus infiltration & frontal lobe lesions by CT scans	Nodal T-cell lymphoma, NOS	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β R	Steroids, acyclovir Dead shortly after
Tanaka et al³²	2/M	Asian	9 y	Multiple nodular lung infiltrates, hemophysis	Pulmonary large cell lymphoma, angiocentric	CD4⁺, HLA-DR⁺	EBV⁺, clonal	TCR-β G	Antiviral agents and interferon Dead shortly after
Kanegane et al³⁰	2/F	Asian	2 y	Generalized lymphadenopathy	Nodal T-cell lymphoma, NOS	CD45RO⁺, CD4⁺	EBV⁺, clonal	TCR-β,γ R	Vincristine and prednisone Clinical remission
	11/M	Asian	5 mo	High fever, hepatosplenomegaly, parotid swelling	Nodal T-cell lymphoma, NOS	CD45RO⁺, CD4⁺	EBV⁺, clonal	TCR-β,γ R	α-interferon, cyclosporin A, etoposide, steroids Dead at 2 y and 4 mo
Ohshima et al²⁷	52/M	Asian	5 y	Lymphadenopathy, hepatosplenomegaly	Nodal T-cell lymphoma, NOS	ND	EBV⁺	ND	Dead, time unspecified
	12/F	Asian	11 mo	Lymphadenopathy, hepatosplenomegaly	NK leukemia	CD56⁺	EBV⁺, clonal	TCR-β,γ G	Dead, time unspecified
	23/F	Asian	6 mo	Hepatosplenomegaly	NK leukemia	CD56⁺	EBV⁺, clonal	TCR-β,γ G	Dead, time unspecified
Kawaguchi et al⁴¹	13/F	Asian	20 mo	Hepatosplenomegaly fever, pancytopenia	Bone marrow lymphocytosis	CD45RO⁺, TCRβ⁺	EBV⁺, clonal	TCR-β,γ G	Dead at 20 months

F4-150

LYG, lymphomatoid granulomatosis. This case in current terminology most probably corresponds to enteropathy-associated T-cell lymphoma.

CAEBV indicates chronic active EBV infection; NS, not specified; TCR, T-cell receptor (R, rearranged; G, germline); NOS, not otherwise specified.

View Large