Table 1.

Pathogenetic insights based on a disease-oriented approach to lymphoma classification

Lymphomas associated with infectious agents
Nasal, cutaneous and systemic NK/T-cell lymphomas	EBV
Adult T-cell leukemia/lymphoma	HTLV1
Marginal zone lymphomas	H pylori, B burgdorferi, C jejuni, Hepatitis C, and others
Primary effusion lymphoma, LBCL associated with multicentric CD	HHV-8/ KSHV
Plasmablastic, Burkitt, DLBCL, CHL	EBV (subset of cases)
Lymphomas with deregulation of apoptosis and survival pathways
Follicular lymphoma	BCL2/IGH@
MALT lymphomas	API2/MALT1 and variants
Lymphomas with deregulation of the cell cycle
Mantle cell lymphoma	CCND1/IGH@
Burkitt's lymphoma	MYC/IGH@ and variants
Lymphomas with deregulation of cell signaling or transcriptional regulation
Anaplastic large cell lymphoma	NPM/ALK and variants
Diffuse large B-cell lymphomas	BCL6, NFκB, Stat6
Lymphomas associated with host susceptibility factors, congenital or acquired
Enteropathy-associated T-cell lymphoma	Genetics, gliadin allergy
Extranodal and systemic EBV + T/NK cell lymphomas	Genetics, host response to EBV
Hepatosplenic T-cell lymphoma	Immunosuppression combined with chronic antigenic stimulation
Lymphomatoid granulomatosis	Partial immune dysfunction and EBV
Burkitt lymphoma	Polyclonal B-cell activation with or without immunosuppression (malaria, HIV)
Posttransplantation and other iatrogenic lymphoproliferative disorders	Iatrogenic immunosuppression

LBCL indicates large B-cell lymphoma.