Table 2. Select differential diagnosis... | American Society of Hematology

Table 2.

Select differential diagnosis in HSTCL and distinguishing features

Diagnosis	Age (median/mean), y	Common symptoms/signs	Morphological findings	Immunohistochemical findings	Genomic findings
HSTCL	32	B symptoms, splenomegaly, cytopenia	Atypical small-intermediate T cells, sinusoidal pattern	CD3⁺, CD2⁺, CD5⁻, CD7^+/−, CD4⁻/CD8⁻, CD56⁺, CD57⁻, EBV⁻	i(7q), trisomy 8, STAT3/STAT5B mutation, CMG mutations
γδ T-LGL⁴¹	62	Neutropenia, anemia splenomegaly +/−	LGL, sinusoidal pattern	CD3⁺, CD2⁺, CD5⁻/dim, CD7⁺, CD4⁻/CD8⁻, CD56^+/−, CD57⁺, EBV⁻	STAT3/STAT5B aberrations
Aggressive NK-cell leukemia⁴²	40	B symptoms, splenomegaly, cytopenia, lymphadenopathy +/−	Medium-large atypical lymphoid cells	Surface CD3⁻, CD2⁺, CD5⁻, CD56⁺, CD16⁺, CD57⁻, EBV⁺	Del(6q), del(11q)
MEITL^66,67	59	GI symptoms	Monomorphic medium-sized cells	CD3⁺, CD5⁻, CD7⁺, CD4⁻, CD8⁺, CD56⁺, CD103^+/−, cytotoxic markers⁺, EBV⁻	Gain in 8q24 (MYC gene), STAT5B mutation
CAEBV⁴⁵	19	B symptoms, cytopenia, splenomegaly, lymphadenopathy	Small-intermediate T cells with no atypia	EBV⁺ in T/NK cells (Asian population) or in B cells (Western population); no phenotypic abnormalities	Rare somatic mutations of perforin

Diagnosis	Age (median/mean), y	Common symptoms/signs	Morphological findings	Immunohistochemical findings	Genomic findings
HSTCL	32	B symptoms, splenomegaly, cytopenia	Atypical small-intermediate T cells, sinusoidal pattern	CD3⁺, CD2⁺, CD5⁻, CD7^+/−, CD4⁻/CD8⁻, CD56⁺, CD57⁻, EBV⁻	i(7q), trisomy 8, STAT3/STAT5B mutation, CMG mutations
γδ T-LGL⁴¹	62	Neutropenia, anemia splenomegaly +/−	LGL, sinusoidal pattern	CD3⁺, CD2⁺, CD5⁻/dim, CD7⁺, CD4⁻/CD8⁻, CD56^+/−, CD57⁺, EBV⁻	STAT3/STAT5B aberrations
Aggressive NK-cell leukemia⁴²	40	B symptoms, splenomegaly, cytopenia, lymphadenopathy +/−	Medium-large atypical lymphoid cells	Surface CD3⁻, CD2⁺, CD5⁻, CD56⁺, CD16⁺, CD57⁻, EBV⁺	Del(6q), del(11q)
MEITL^66,67	59	GI symptoms	Monomorphic medium-sized cells	CD3⁺, CD5⁻, CD7⁺, CD4⁻, CD8⁺, CD56⁺, CD103^+/−, cytotoxic markers⁺, EBV⁻	Gain in 8q24 (MYC gene), STAT5B mutation
CAEBV⁴⁵	19	B symptoms, cytopenia, splenomegaly, lymphadenopathy	Small-intermediate T cells with no atypia	EBV⁺ in T/NK cells (Asian population) or in B cells (Western population); no phenotypic abnormalities	Rare somatic mutations of perforin

CAEBV, chronic active EBV disease; CMG, chromatin-modifying gene; MEITL, monomorphic epitheliotropic intestinal T-cell lymphoma.