Table 3. Review of previously reported... | American Society of Hematology

Table 3.

Review of previously reported cases of fulminant EBV⁺ T-cell LPD following acute EBV infection

Reference	Age/ sex	Ethnic origin	Clinical presentation	Histopathologic features	Phenotype	EBV status	TCR status	Therapy and outcome
Su et al³⁹	13/M	Asian	Hepatosplenomegaly, jaundice, pleural adhesion	Nodal 1 immunoblastic lymphoma	CD8⁺	EBV⁺	TCR-β R	CHOP; dead at 3 mo
	7/M	Asian	Adenopathy, hepatosplenomegaly	Nodal ALILD-like T cell lymphoma	CD8⁺	EBV⁺	TCR-β R	CHOP, bleomycin, precarbazine; dead at 13 mo
Mori et al³⁷	2/M	Asian	IM followed by hepatic failure, sepsis, pancytopenia	Systemic atypical lymphoid infiltrates with necrosis	CD8⁺	EBV⁺, clonal	ND	Antivirus, interferon-alpha, steroids; dead at 31 d
Gaillard et al³⁶	7/F	Unspecified	IM followed by persistent fever, adenopathy, VAHS	Systemic atypical lymphoid infiltrates with necrosis	CD8⁺	EBV⁺	TCR-β & γ R	Steroids, acyclovir, VP16; dead at 1 y
Chan et al³³	3/F	Asian	Fever, adenopathy, hepatosplenomegaly	Large granular lymphocytosis in peripheral blood, tissue morphology not specified	CD8⁺	EBV⁺, clonal	TCR-β R	Antibiotics; dead at 11 d
Craig et al³⁴	20 mo/ F	Unspecified	Fever, generalized erythematous skin rash, hepatosplenomegaly	Splenic and mesenteric nodal inv by T-cell lymphoma NOS	Uninterpretable	EBV⁺, clonal	TCR-β R	Supportive therapy; dead at 25 d
Tazawa et al⁴⁰	1/M	Asian	Fever, tonsillitis, cervical adenopathy hepatosplenomegaly, grancytopenia	Systemic lymphoid infiltrates, lacking cytologic atypia	CD8⁺	EBV⁺	ND	Supportive therapy; dead at 3 d
Noma et al³⁸	1/F	Asian	Fever, pancytopenia, hepatosplenomegaly	Not specified, bone marrow analyzed	CD4⁺ & CD8⁺	EBV⁺, clonal	TCR-β & γ R	VP16 therapy; relapse at 15 mo, no further follow-up
Dolezal et al³⁵	24/M	Asian	Headache, fever, night sweats, hepatosplenomegaly, elevated liver enzymes, VAHS	Systemic lymphoid infiltrates, polymorphosis, lacking cytologic atypia	CD56⁺, CD8⁺, CD3⁻ (true NK)	EBV⁺, clonal	TCR-β G	Antibiotics, dead at 1 mo
Kawaguchi et al⁴¹	5/M	Asian	Fever, hepatosplenomegaly, pancytopenia, VAHS	Liver and bone marrow small lymphoid infiltrates	CD45RO, TCR-β⁺	EBV⁺, clonal	TCR-β γG	Dead at 3 mo
	1/M	Asian	Fever, hepatosplenomegaly, pancytopenia, VAHS	Depleted lymph nodes, hypocellular BM with relative lymphocytosis, not further specified	CD45RO, TCR-β	EBV⁺, dorsal	TCR-β γ G	Dead at 1 mo

Reference	Age/ sex	Ethnic origin	Clinical presentation	Histopathologic features	Phenotype	EBV status	TCR status	Therapy and outcome
Su et al³⁹	13/M	Asian	Hepatosplenomegaly, jaundice, pleural adhesion	Nodal 1 immunoblastic lymphoma	CD8⁺	EBV⁺	TCR-β R	CHOP; dead at 3 mo
	7/M	Asian	Adenopathy, hepatosplenomegaly	Nodal ALILD-like T cell lymphoma	CD8⁺	EBV⁺	TCR-β R	CHOP, bleomycin, precarbazine; dead at 13 mo
Mori et al³⁷	2/M	Asian	IM followed by hepatic failure, sepsis, pancytopenia	Systemic atypical lymphoid infiltrates with necrosis	CD8⁺	EBV⁺, clonal	ND	Antivirus, interferon-alpha, steroids; dead at 31 d
Gaillard et al³⁶	7/F	Unspecified	IM followed by persistent fever, adenopathy, VAHS	Systemic atypical lymphoid infiltrates with necrosis	CD8⁺	EBV⁺	TCR-β & γ R	Steroids, acyclovir, VP16; dead at 1 y
Chan et al³³	3/F	Asian	Fever, adenopathy, hepatosplenomegaly	Large granular lymphocytosis in peripheral blood, tissue morphology not specified	CD8⁺	EBV⁺, clonal	TCR-β R	Antibiotics; dead at 11 d
Craig et al³⁴	20 mo/ F	Unspecified	Fever, generalized erythematous skin rash, hepatosplenomegaly	Splenic and mesenteric nodal inv by T-cell lymphoma NOS	Uninterpretable	EBV⁺, clonal	TCR-β R	Supportive therapy; dead at 25 d
Tazawa et al⁴⁰	1/M	Asian	Fever, tonsillitis, cervical adenopathy hepatosplenomegaly, grancytopenia	Systemic lymphoid infiltrates, lacking cytologic atypia	CD8⁺	EBV⁺	ND	Supportive therapy; dead at 3 d
Noma et al³⁸	1/F	Asian	Fever, pancytopenia, hepatosplenomegaly	Not specified, bone marrow analyzed	CD4⁺ & CD8⁺	EBV⁺, clonal	TCR-β & γ R	VP16 therapy; relapse at 15 mo, no further follow-up
Dolezal et al³⁵	24/M	Asian	Headache, fever, night sweats, hepatosplenomegaly, elevated liver enzymes, VAHS	Systemic lymphoid infiltrates, polymorphosis, lacking cytologic atypia	CD56⁺, CD8⁺, CD3⁻ (true NK)	EBV⁺, clonal	TCR-β G	Antibiotics, dead at 1 mo
Kawaguchi et al⁴¹	5/M	Asian	Fever, hepatosplenomegaly, pancytopenia, VAHS	Liver and bone marrow small lymphoid infiltrates	CD45RO, TCR-β⁺	EBV⁺, clonal	TCR-β γG	Dead at 3 mo
	1/M	Asian	Fever, hepatosplenomegaly, pancytopenia, VAHS	Depleted lymph nodes, hypocellular BM with relative lymphocytosis, not further specified	CD45RO, TCR-β	EBV⁺, dorsal	TCR-β γ G	Dead at 1 mo

IM indicates infectious mononucleosis; VAHS, virus-associated hepatophagocytic syndrome; TCR, T-cell receptor (R, rearranged; G, germline); AILD, angioimmunoblastic lymphadenopathy; NOS, not otherwise specified; ND, not done.

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