Table 2.

Differential diagnosis of large B-cell lymphomas with intravascular presentation

	IVLBL classic variant	IVLBL HPS–associated variant	IVLBL cutaneous variant	EBV + large B-cell lymphoma with intravascular presentation [Ref. 4–8]	HHV8 + , EBV + / − large B-cell lymphoma with intravascular presentation [9–15]
Median age	67 years	67 years	59 years	57 years	39 years
Gender	M = F	M = F	F > M	M > F	M > F
Ethnicity	Western	Asian	Western	Mostly Asian	Western
Immunodeficiency association	No	No	No	HIV or immunosuppressive treatment for autoimmune disorders	HIV, post-transplant setting
Clinical presentation	Fever, organ-specific local symptoms, CNS and cutaneous involvement, B symptoms, multiorgan failure	Multiorgan failure, hepatosplenomegaly, pancytopenia	Single or multiple lesions of the skin with negative systemic staging	Fever, fatigue, weight loss, organ-specific local symptoms	Fever, weight loss, Kaposi sarcoma lesions, hepatosplenomegaly, pleural effusions
Organs involved	Widely disseminated, frequent SNC and skin involvement	Bone marrow, liver, spleen	Skin	Variable, localized disease (50%)	Spleen, liver, pleural cavities, skin
Laboratory findings	Anaemia, increased LDH	Anaemia, thrombocytopenia, increased LDH	Normal leukocyte and platelet counts	Anaemia, pancytopenia	Pancytopenia, anaemia, thrombocytopenia
Cytology/ Immunophenotype	Large, atypical cells with prominent nucleoli/ CD20 + , MUM1 + , BCL6-/ + , CD10-/ + , CD5 + (38%)			Large, atypical cells with prominent nucleoli / CD20 + , MUM1 + , BCL6-/ + , CD10-/ + , CD5 + (50%)	Large, atypical cells with plasmablastic morphology/ CD45 + , CD20-, MUM1 +
HPS	-	+	-	+ (33%) or -	-
EBV/HHV8	-/-	-/-	-/-	+ / −	+ (62%)/ +

CNS central nervous system, EBV Epstein-Barr virus, F female, HHV8 human herpes virus 8, HIV human immunodeficiency virus, HPS haemophagocytic syndrome, IVLBL intravascular large B-cell lymphoma, LDH lactate dehydrogenase, M male